Materiały źródłowe

• #1 Hypereosinophilic Syndrome

  https://www.aaaai.org/conditions-treatments/related-conditions/hypereosinophilic-syndrome

  Most people have less than 500 eosinophils/microliter in their blood. People with HES usually have more than 1,500 eosinophils/microliter in their blood for 6 months or more, and the cause cannot be identified. […] Symptoms HES can happen at any age, although it is more common in adults. People with HES may suffer from a wide variety of symptoms, depending upon which parts of the body are affected. These symptoms include: Skin rashes such as urticaria or angioedema, Dizziness, Memory loss or confusion, Cough, Shortness of breath, Fatigue, Fever, Mouth sores. […] The prognosis of HES depends upon the extent of any organ damage. In very severe cases, HES may be fatal, but there is hope. Survival rates have improved greatly. In 1975, only 12% of HES patients survived three years. Today, more than 80% of HES patients survive five years or more.

• #2 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Hypereosinophilic syndrome (HES) encompasses a rare and complex group of heterogeneous disorders characterized by persistent and substantial elevations in eosinophil levels and mediators leading to tissue infiltration and damage. […] According to a retrospective analysis of patients between 2001 to 2006, approximately 37% experienced dermatologic signs and symptoms. Around 25% of these were pulmonary, followed by 14% gastrointestinal, 5% cardiac, and 4% neurologic. […] Dermatologic manifestations associated with HES encompass a spectrum of conditions, including eczema, erythroderma, skin lichenification, dermographism, urticaria, and angioedema. Notably, prominent cutaneous symptoms may indicate L-HES, warranting suspicion of potential cutaneous T-cell lymphoma. Initial signs of HES may manifest as skin lesions on the trunk and extremities, presenting as pruritic erythematous macules, papules, plaques, wheals, and nodules.

• #3 Hypereosinophilic Syndrome: What It Is, Causes, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/22541-hypereosinophilic-syndrome

  Hypereosinophilic syndrome happens when eosinophilia accelerates, speeding up eosinophil production and increasing the number of eosinophils. This eosinophil overload can damage your organs, including your heart, lungs, skin and nervous system. Left untreated, hypereosinophilic syndrome can be life-threatening. Fortunately, prompt diagnosis and treatment mean more than 80% of those diagnosed with hypereosinophilic syndrome are alive five years after diagnosis. […] The most common HES symptom is a rash. Hypereosinophilic symptoms correlate (match to) to the area of your body affected by high eosinophil levels. For example, if your heart has an unusually high level of eosinophils, you might have symptoms of congestive heart failure, cardiomyopathy, myocarditis and pericardial effusion. Likewise, if the eosinophils are congregating in your lungs, you might have recurring (repeating) upper respiratory infections, coughing and difficulty breathing. […] Generally speaking, the sooner your HES is diagnosed and treated, the less likely you are to have lasting problems.

• #4 Hypereosinophilic syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hypereosinophilic-syndrome/symptoms-causes/syc-20352854

  Early symptoms of HES may include fatigue, cough, breathlessness, muscle pain, rash and fever. […] The most common targets are the skin, lungs, digestive tract, heart, blood and nervous system. Untreated, HES can become life-threatening.

• #5 Hypereosinophilic Syndrome: Symptoms, Diagnosis, Treatment, and More

  https://www.healthline.com/health/hypereosinophilic-syndrome

  Eosinophils are an important part of your immune system, but too many of these white blood cells can result in rare blood disorders, collectively known as hypereosinophilic syndrome. […] In people with HES, these extra eosinophils start damaging nearby tissue and organs, resulting in a range of potential symptoms. […] HES symptoms can vary greatly from person to person, depending on the organs and body systems involved. […] Potential early symptoms of HES include: coughing, fatigue, wheezing, shortness of breath, fever, skin rashes, dizziness, memory loss, confusion, mouth sores, weight loss. […] Later symptoms will also depend on the organs and body systems involved. […] Theres currently no cure for HES, but treating the condition can help prevent organ damage and slow disease progression. […] The main goal of HES treatment is lowering the number of eosinophils in your body. […] While theres no cure for HES, treatment can help prevent organ damage and disease progression by keeping eosinophils at more manageable levels.

• #6 Hypereosinophilic Syndrome (HES): Types, Symptoms, & More

  https://www.health.com/hypereosinophilic-syndrome-8546797

  Symptoms of HES vary depending on the organ system affected but commonly include skin rash, fever, fatigue, muscle aches, weakness, and weight loss. […] Symptoms common with all types of HES include: Skin rash, which may be red, itchy bumps (urticaria/hives) or swollen lumps under the skin (angioedema), Fatigue, Fever, Muscle aches, Weakness, Loss of appetite and/or unintentional weight loss, Cough, Shortness of breath, Memory problems, Confusion. […] Treatment for hypereosinophilic syndrome focuses on decreasing high eosinophil levels in your blood, reducing inflammation to prevent organ damage, and slowing disease progression. […] Untreated HES can cause extensive damage to the heart, lungs, gastrointestinal tract, and other organs, increasing your risk of life-threatening complications.

• #7 What Is Hypereosinophilic Syndrome?

  https://www.icliniq.com/articles/blood-health/hypereosinophilic-syndrome

  Hypereosinophilic syndrome (HES) is a rare condition that happens when eosinophilia accelerates, speeding up eosinophil production and increasing the number of eosinophils. This eosinophil overload can damage the organs, including the heart, lungs, skin, and nervous system. If left untreated, the hypereosinophilic syndrome can be life-threatening. Most HES patients are over 20 years of age, but children can also be affected. Most variants of HES are considered benign (non-cancerous), but a subset is known to be myeloproliferative neoplasms, which can turn into cancer. […] Common and early HES symptoms include fatigue, cough, shortness of breath, myalgia, skin rash, and fever. Symptoms of hypereosinophilia correlate with areas of the body affected by high levels of eosinophils. Such as congestive heart failure, cardiomyopathy, myocarditis, and pericardial effusion. The accumulation of eosinophils in the lungs can cause recurrent upper respiratory tract infections, coughing, and dyspnea.

• #8

  https://link.springer.com/article/10.1007/s40629-022-00221-w

  Sustained elevation of eosinophils above 5109/l in peripheral blood (PB) should prompt further investigation. […] The diagnosis of HES is made when organ infiltration with consecutive dysfunction is diagnosed in persistent eosinophilia after exclusion of other causes. […] Clinical presentation in HES depends on the number of involved organs and the extent of organ involvement. Frequently affected organs include skin, sinuses, lungs, heart, gastrointestinal tract, or nervous system; in principle, any organ may be involved. […] While in some patients eosinophilia is diagnosed by chance during a routine check-up of the PB in asymptomatic or only mildly symptomatic patients (e.g., itching, urticaria), in other cases severe, sometimes life-threatening courses occur due to organ infiltration and consecutive dysfunction.

• #9 Pathology Outlines – Idiopathic hypereosinophilic syndrome

  https://www.pathologyoutlines.com/topic/myeloproliferativehypereosinophilicsyndrome.html

  Idiopathic hypereosinophilic syndrome is a disorder defined by peripheral blood eosinophilia (absolute eosinophil count ≥ 1.5 x 10^9/L) for at least 6 months with organ damage / dysfunction attributable to tissue hypereosinophilic infiltrate per biopsy and no discernible underlying etiology. […] Onset of symptoms is often insidious with eosinophilia being detected incidentally; however, some patients initially present with severe and life threatening problems due to the rapid progression of cardiovascular and neurologic complications. […] Dermatologic symptoms were most common, followed by pulmonary, gastrointestinal, cardiac and lastly neurologic. […] Cardiac damage, valvular fibrosis, thromboembolism. […] Slowly progressive clinical course with death occurring due to cardiac damage in some cases.

• #10 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Hypereosinophilic syndrome (HES) encompasses a rare and complex group of heterogeneous disorders characterized by persistent and substantial elevations in eosinophil levels and mediators leading to tissue infiltration and damage. […] According to a retrospective analysis of patients between 2001 to 2006, approximately 37% experienced dermatologic signs and symptoms. Around 25% of these were pulmonary, followed by 14% gastrointestinal, 5% cardiac, and 4% neurologic. […] Dermatologic manifestations associated with HES encompass a spectrum of conditions, including eczema, erythroderma, skin lichenification, dermographism, urticaria, and angioedema. Notably, prominent cutaneous symptoms may indicate L-HES, warranting suspicion of potential cutaneous T-cell lymphoma. Initial signs of HES may manifest as skin lesions on the trunk and extremities, presenting as pruritic erythematous macules, papules, plaques, wheals, and nodules.

• #11 Hypereosinophilic syndrome

  https://dermnetnz.org/topics/hypereosinophilic-syndrome

  Hypereosinophilic syndrome usually presents with fever, weight loss, fatigue, and rash. […] A rash is present in over 50% of patients, but is non-specific in appearance. Most commonly, red swollen itchy nodules (lumps) have been described. It can also resemble urticaria (hives). […] An enlarged liver and spleen is often present indicating liver and spleen involvement. The lungs, kidneys, heart and nervous system can be affected. […] The overall 5-year survival for patients with hypereosinophilic syndrome is 80% and the cause of death is usually heart failure.

• #12 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Hypereosinophilic syndrome (HES) encompasses a rare and complex group of heterogeneous disorders characterized by persistent and substantial elevations in eosinophil levels and mediators leading to tissue infiltration and damage. […] According to a retrospective analysis of patients between 2001 to 2006, approximately 37% experienced dermatologic signs and symptoms. Around 25% of these were pulmonary, followed by 14% gastrointestinal, 5% cardiac, and 4% neurologic. […] Dermatologic manifestations associated with HES encompass a spectrum of conditions, including eczema, erythroderma, skin lichenification, dermographism, urticaria, and angioedema. Notably, prominent cutaneous symptoms may indicate L-HES, warranting suspicion of potential cutaneous T-cell lymphoma. Initial signs of HES may manifest as skin lesions on the trunk and extremities, presenting as pruritic erythematous macules, papules, plaques, wheals, and nodules.

• #13 Hypereosinophilic syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hypereosinophilic_syndrome

  Symptoms Fatigue, breathlessness, cough, muscle pain, fever, and rash. […] Hypereosinophilic syndrome can manifest in many different ways from nonspecific symptoms and fatigue to neurological impairment and endomyocardial fibrosis, which may be fatal. […] Patients can develop a range of nonspecific symptoms, including fever, diarrhea, rash, angioedema, weakness, exhaustion, coughing, and dyspnea. […] The common and non-specific cutaneous manifestations are either erythematous, itchy papules and nodules that resemble eczema, or urticarial and angioedematous lesions. […] Cardiac involvement typically progresses through three phases. Rarely, the early necrotic stage involving the endo-myocardium manifests as acute heart failure. In most cases, however, there are no symptoms. A thrombotic stage ensues after this one, during which thrombi form in the cardiac chambers along the injured endocardium and may separate, resulting in peripheral emboli. Endomyocardial fibrosis causes irreversible restrictive cardiomyopathy in the final stage of fibrosis, and damage to the atrioventricular valves may cause more acute presentations of congestive heart failure.

• #14 Hypereosinophilic syndrome – wikidoc

  https://www.wikidoc.org/index.php/Hypereosinophilic_syndrome

  Symptoms of hypereosinophilic syndrome may include the following: Shortness of breath, Skin rash, Cough, Diarrhea, Myalgias, Fatigue, Weight-loss. […] Patients with hypereosinophilic syndrome usually have a normal appearance. Physical examination may be remarkable for: Skin rash, Thickening of the skin (lichenification), Eczema (flexural areas), Dermographism, Low-grade fever, Raynaud phenomenon, Wheezing. […] Findings related with poor prognosis in hypereosinophilic syndrome, include: presence of anemia, thrombocytopenia, white blood cell count greater than 100,000 cells/cm3, abnormal marrow and/or basophils, elevated serum levels of vitamin B12, serum tryptase, and abnormal levels of leukocyte alkaline phosphatase.

• #15 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Hypereosinophilic syndrome (HES) encompasses a rare and complex group of heterogeneous disorders characterized by persistent and substantial elevations in eosinophil levels and mediators leading to tissue infiltration and damage. […] According to a retrospective analysis of patients between 2001 to 2006, approximately 37% experienced dermatologic signs and symptoms. Around 25% of these were pulmonary, followed by 14% gastrointestinal, 5% cardiac, and 4% neurologic. […] Dermatologic manifestations associated with HES encompass a spectrum of conditions, including eczema, erythroderma, skin lichenification, dermographism, urticaria, and angioedema. Notably, prominent cutaneous symptoms may indicate L-HES, warranting suspicion of potential cutaneous T-cell lymphoma. Initial signs of HES may manifest as skin lesions on the trunk and extremities, presenting as pruritic erythematous macules, papules, plaques, wheals, and nodules.

• #16 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Hypereosinophilic syndrome (HES) encompasses a rare and complex group of heterogeneous disorders characterized by persistent and substantial elevations in eosinophil levels and mediators leading to tissue infiltration and damage. […] According to a retrospective analysis of patients between 2001 to 2006, approximately 37% experienced dermatologic signs and symptoms. Around 25% of these were pulmonary, followed by 14% gastrointestinal, 5% cardiac, and 4% neurologic. […] Dermatologic manifestations associated with HES encompass a spectrum of conditions, including eczema, erythroderma, skin lichenification, dermographism, urticaria, and angioedema. Notably, prominent cutaneous symptoms may indicate L-HES, warranting suspicion of potential cutaneous T-cell lymphoma. Initial signs of HES may manifest as skin lesions on the trunk and extremities, presenting as pruritic erythematous macules, papules, plaques, wheals, and nodules.

• #17 Hypereosinophilic Syndrome and T-Cell Lymphoma: Which Comes First? | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-hypereosinophilic-syndrome-t-cell-lymphoma-articulo-S0001731023005902

  Hypereosinophilic syndrome (HES) is defined by a concentration of eosinophils in peripheral blood greater than 1500cells/L, accompanied by organ damage or dysfunction not attributable to other causes. […] Close follow-up of these patients is essential, as 10-20% may progress to cutaneous T-cell lymphoma (CTCL) with a poor prognosis. […] A 67-year-old man attended the clinic with symptoms of 3 months standing of polyarthralgia, multiple migratory bilateral pulmonary infiltrates, generalized highly itchy maculopapular skin rash, and constitutional syndrome with asthenia, anorexia, and weight loss. […] Eosinophilia has an important prognostic role in CTCL, with median survival 3 years lower in patients with eosinophil levels above 700/L. Some authors have considered eosinophilia on diagnosis as the only prognostic variable associated with disease progression and disease-related death.

• #18 COVID-19 Pneumonia or Hypereosinophilic Syndrome? | Serin | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/3587/2919

  Hypereosinophilic syndromes (HESs) are a group of disorders characterized by pathological proliferation of eosinophils. […] Fatigue, cough, shortness of breath, myalgia, angioedema, rhinitis, rash and fever are the most common symptoms of hypereosinophilia. The most common clinical findings in the follow-up of patients with hypereosinophilia are due to dermatological involvement (69%). Then there are pulmonary (44%) and gastrointestinal (38%) findings. […] Lung involvement can be seen in 40-60% of cases in HES. The most common respiratory symptom is the chronic and persistent cough. Lung involvement results in sputum, wheezing, and shortness of breath, which increases the suspicion of bronchial hyperreactivity. […] When cardiac involvement occurs, the most common form is endomyocardial fibrosis. Progressive scarring, especially in the posterior mitral valve, mostly limits the movement of the valve.

• #19 Hypereosinophilic syndrome presenting as coagulopathy | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-022-00666-2

  Hypereosinophilic syndrome (HES) is an extremely uncommon group of disorders. It rarely presents with coagulopathy without cardiac involvement. […] HES typically presents with symptoms of weakness and fatigue, cough, dyspnea, myalgia, angioedema, rash, or fever. […] The most common organ systems involved with presentation of HES are dermatologic (69%), pulmonary (44%), and gastrointestinal (38%). […] Thromboembolic events are thought to occur in 25% of patients with cardiac involvement of HES, which has previously been attributed to endocardial damage leading to mural thrombi. […] In patients with an unexplained coagulopathy and eosinophilia, eosinophilic disorders such as HES should be considered. […] Our patient highlights the importance of having an increased index of suspicion for HES in patients presenting with eosinophilia and thrombosis. Rapid diagnosis of HES and utilization of steroid-sparing agents such as anti-IL-5 therapies could help prevent further end-organ damage, treat an underlying mechanism for associated coagulopathy, and avoid long-term side effects of corticosteroid use.

• #20 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Pulmonary manifestations of HES include asthma-like symptoms, including dyspnea, cough, and wheezing. Abnormal findings such as patchy ground-glass infiltrates and consolidation within the pulmonary parenchyma are observed on chest radiography or computed tomography (CT). […] Gastrointestinal involvement includes colitis or enteritis, eosinophilic gastritis, chronic active hepatitis, focal hepatic lesions, eosinophilic cholangitis, and Budd-Chiari syndrome. These may present as dysphagia, weight loss, abdominal pain, nausea, vomiting, and diarrhea. […] Cardiac manifestations exhibit a higher incidence in FIP1L1-PDGFRA-associated HES. This phenomenon arises from the infiltration of eosinophils into the myocardium, leading to myocardial necrosis due to the release of toxic cationic proteins and enhanced leukocyte recruitment or activation.

• #21

  https://link.springer.com/article/10.1007/s40629-022-00221-w

  Cardiac involvement is particularly problematic, with endomyocardial fibrosis and intracardiac thrombi (Lfflers endocarditis), thromboembolic complications, peri/myocarditis, pericardial effusion, and heart failure. […] Pulmonary involvement is most commonly manifested by bronchial asthma, pulmonary infiltrates, pleural effusion, or pulmonary fibrosis. […] Gastrointestinal involvement includes esophagitis, gastroenteritis, serositis, ascites, liver dysfunction, and splenomegaly. […] The extent and number of organs affected allows conclusions to be drawn about the genesis of the eosinophilia. In clonal eosinophilia, there is often only isolated splenomegaly or involvement of rather few other organs, for example heart or skin. A high number of affected organs and involvement of the upper/lower respiratory tract and/or the gastrointestinal tract are more indicative of the presence of HES.

• #22 Orphanet: Hypereosinophilic syndrome

  https://www.orpha.net/en/disease/detail/168956

  Hypereosinophilic syndrome (HES) constitutes a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia and/or tissue eosinophilia associated with a wide range of clinical manifestations reflecting eosinophil-induced tissue/organ damage. […] Target-organ damage mediated by eosinophils is highly variable among patients, and consists of dermatological involvement (urticaria, eczema, angioedema, pruriginous papules, nodules, erythroderma) in more than 50% of cases, followed by involvement of lungs (cough, breathlessness and wheezing) and digestive tract (nausea, vomiting, abdominal pain, diarrhea, ascites) in roughly 40%. Cardiac involvement is less frequent, but must be recognized early due to irreversible and life-threatening complications such as acute myocarditis, intraventricular thrombus, endomyocardial fibrosis and valve thickening and/or destruction. Constitutional symptoms of fever, myalgia and fatigue may occur. Other common complications include central or peripheral nervous system involvement, hepato- and/or splenomegaly, and coagulation disorders. […] Prognosis has improved significantly since defining HES, and currently depends on the development of irreversible endomyocardial fibrosis, as well as eventual malignant transformation of myeloid or lymphoid cells.

• #23 Hypereosinophilic syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hypereosinophilic_syndrome

  Both the peripheral (polyneuropathy) and central (diffuse encephalopathy) nervous systems may be affected by neurological manifestations. Disorientation, memory loss, and altered behavior and cognitive function are the symptoms of diffuse encephalopathy. Symptoms of peripheral neuropathies can include mixed sensory and motor complaints, symmetric or asymmetric sensory alterations, or pure motor deficits. […] When there are no radiological abnormalities, lung involvement can vary from a persistent dry cough and/or bronchial hyperreactivity to restrictive disease with pulmonary infiltrates. […] Abdominal pain, diarrhea, nausea, and vomiting are a few examples of gastrointestinal symptoms. […] If left untreated, HES is progressive and fatal.

• #24 Hypereosinophilic syndrome presenting as coagulopathy | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-022-00666-2

  Hypereosinophilic syndrome (HES) is an extremely uncommon group of disorders. It rarely presents with coagulopathy without cardiac involvement. […] HES typically presents with symptoms of weakness and fatigue, cough, dyspnea, myalgia, angioedema, rash, or fever. […] The most common organ systems involved with presentation of HES are dermatologic (69%), pulmonary (44%), and gastrointestinal (38%). […] Thromboembolic events are thought to occur in 25% of patients with cardiac involvement of HES, which has previously been attributed to endocardial damage leading to mural thrombi. […] In patients with an unexplained coagulopathy and eosinophilia, eosinophilic disorders such as HES should be considered. […] Our patient highlights the importance of having an increased index of suspicion for HES in patients presenting with eosinophilia and thrombosis. Rapid diagnosis of HES and utilization of steroid-sparing agents such as anti-IL-5 therapies could help prevent further end-organ damage, treat an underlying mechanism for associated coagulopathy, and avoid long-term side effects of corticosteroid use.

• #25 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Pulmonary manifestations of HES include asthma-like symptoms, including dyspnea, cough, and wheezing. Abnormal findings such as patchy ground-glass infiltrates and consolidation within the pulmonary parenchyma are observed on chest radiography or computed tomography (CT). […] Gastrointestinal involvement includes colitis or enteritis, eosinophilic gastritis, chronic active hepatitis, focal hepatic lesions, eosinophilic cholangitis, and Budd-Chiari syndrome. These may present as dysphagia, weight loss, abdominal pain, nausea, vomiting, and diarrhea. […] Cardiac manifestations exhibit a higher incidence in FIP1L1-PDGFRA-associated HES. This phenomenon arises from the infiltration of eosinophils into the myocardium, leading to myocardial necrosis due to the release of toxic cationic proteins and enhanced leukocyte recruitment or activation.

• #26 Hypereosinophilic syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hypereosinophilic_syndrome

  Both the peripheral (polyneuropathy) and central (diffuse encephalopathy) nervous systems may be affected by neurological manifestations. Disorientation, memory loss, and altered behavior and cognitive function are the symptoms of diffuse encephalopathy. Symptoms of peripheral neuropathies can include mixed sensory and motor complaints, symmetric or asymmetric sensory alterations, or pure motor deficits. […] When there are no radiological abnormalities, lung involvement can vary from a persistent dry cough and/or bronchial hyperreactivity to restrictive disease with pulmonary infiltrates. […] Abdominal pain, diarrhea, nausea, and vomiting are a few examples of gastrointestinal symptoms. […] If left untreated, HES is progressive and fatal.

• #27 Orphanet: Hypereosinophilic syndrome

  https://www.orpha.net/en/disease/detail/168956

  Hypereosinophilic syndrome (HES) constitutes a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia and/or tissue eosinophilia associated with a wide range of clinical manifestations reflecting eosinophil-induced tissue/organ damage. […] Target-organ damage mediated by eosinophils is highly variable among patients, and consists of dermatological involvement (urticaria, eczema, angioedema, pruriginous papules, nodules, erythroderma) in more than 50% of cases, followed by involvement of lungs (cough, breathlessness and wheezing) and digestive tract (nausea, vomiting, abdominal pain, diarrhea, ascites) in roughly 40%. Cardiac involvement is less frequent, but must be recognized early due to irreversible and life-threatening complications such as acute myocarditis, intraventricular thrombus, endomyocardial fibrosis and valve thickening and/or destruction. Constitutional symptoms of fever, myalgia and fatigue may occur. Other common complications include central or peripheral nervous system involvement, hepato- and/or splenomegaly, and coagulation disorders. […] Prognosis has improved significantly since defining HES, and currently depends on the development of irreversible endomyocardial fibrosis, as well as eventual malignant transformation of myeloid or lymphoid cells.

• #28 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Pulmonary manifestations of HES include asthma-like symptoms, including dyspnea, cough, and wheezing. Abnormal findings such as patchy ground-glass infiltrates and consolidation within the pulmonary parenchyma are observed on chest radiography or computed tomography (CT). […] Gastrointestinal involvement includes colitis or enteritis, eosinophilic gastritis, chronic active hepatitis, focal hepatic lesions, eosinophilic cholangitis, and Budd-Chiari syndrome. These may present as dysphagia, weight loss, abdominal pain, nausea, vomiting, and diarrhea. […] Cardiac manifestations exhibit a higher incidence in FIP1L1-PDGFRA-associated HES. This phenomenon arises from the infiltration of eosinophils into the myocardium, leading to myocardial necrosis due to the release of toxic cationic proteins and enhanced leukocyte recruitment or activation.

• #29 Hypereosinophilic Syndrome: What It Is, Causes, Complications

  https://www.verywellhealth.com/hypereosinophilic-syndrome-7564454

  Hypereosinophilic syndromes affect organs other than the skin. HES in the heart, lungs, stomach, intestines, and other organs can lead to organ dysfunction. Almost half (44%) of people with HES have lung symptoms, and 38% have intestinal issues. Eventually, about 20% develop heart issues. […] Other common symptoms of hypereosinophilic syndromes include weakness and fatigue, cough, trouble breathing, muscle aches and pain, swelling of the deeper layers of the skin, caused by a buildup of fluid, fever, and a runny nose, called rhinitis. […] HES prognosis depends on what’s causing the disease. It cannot be cured, but medications can control most types. How the disease responds to these treatments plays a significant role in how the condition plays out. […] Prognosis depends on what organs the eosinophils are attacking and how much damage they’ve done. If the damage is already severe, it can’t be undone, and HES may be fatal. The most common cause of death in cases of HES is heart failure. Today, more than 80% of HES patients survive five years or more.

• #30 Idiopathic Hypereosinophilic Syndrome with Multiple Organ Involvement

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7983584/

  Idiopathic hypereosinophilic syndrome is a rare disease which is diagnosed after excluding other conditions. The syndrome is characterized by multiple organ involvement including the heart, nervous system, lungs, and gastrointestinal tract. The disease is suspected if there is peripheral blood eosinophilia and no clear etiology. Patients who do not respond to corticosteroids can be treated with imatinib, immunomodulatory agents, myelosuppressive therapy, or mepolizumab. Alemtuzumab can be considered in severe cases that are unresponsive to other therapies. Our patient responded well to steroids appreciated by improvement in clinical symptoms and decrease in eosinophil count. […] Eosinophilic myocarditis is a major cause of morbidity and mortality among patients with HES. It is characterized by myocardial infiltration with eosinophils and lymphocytes followed by myocardial necrosis. Common cardiac presentations include heart failure, chest pain, arrhythmia, and cardiac thrombi.

• #31 Hypereosinophilic Syndrome: What It Is, Causes, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/22541-hypereosinophilic-syndrome

  Hypereosinophilic syndrome happens when eosinophilia accelerates, speeding up eosinophil production and increasing the number of eosinophils. This eosinophil overload can damage your organs, including your heart, lungs, skin and nervous system. Left untreated, hypereosinophilic syndrome can be life-threatening. Fortunately, prompt diagnosis and treatment mean more than 80% of those diagnosed with hypereosinophilic syndrome are alive five years after diagnosis. […] The most common HES symptom is a rash. Hypereosinophilic symptoms correlate (match to) to the area of your body affected by high eosinophil levels. For example, if your heart has an unusually high level of eosinophils, you might have symptoms of congestive heart failure, cardiomyopathy, myocarditis and pericardial effusion. Likewise, if the eosinophils are congregating in your lungs, you might have recurring (repeating) upper respiratory infections, coughing and difficulty breathing. […] Generally speaking, the sooner your HES is diagnosed and treated, the less likely you are to have lasting problems.

• #32 Hypereosinophilic Symptoms | National Jewish Health

  https://www.nationaljewish.org/conditions/hypereosinophilic-syndromes/symptoms

  Symptoms of HES depend on which organs are involved. […] Some typical symptoms include: a skin rash, cough and breathlessness, abdominal pain, diarrhea/vomiting, and weight loss. […] Cardiovascular problems may include: cardiac arrhythmia (abnormal heartbeat), cardiac dysfunction causing shortness of breath or accumulation of fluid that causes swelling (edema), and blood clots in the heart. […] In its early stages, the symptoms of HES can be silent and not easily detected.

• #33 Hypereosinophilic Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/202030-overview

  Hypereosinophilic syndrome (HES) is a myeloproliferative disorder (MPD) characterized by persistent eosinophilia that is associated with damage to multiple organs. […] Hypereosinophilic syndrome carries a variable prognosis. It is a chronic and progressive disorder that is potentially fatal if left untreated. […] The most serious complication of hypereosinophilic syndrome is cardiac involvement, which can lead to myocardial fibrosis, chronic heart failure, and death. […] Patients with HES with the PDGFRA mutation have a very high incidence of cardiac involvement and carry a poor prognosis without therapy. […] Hypereosinophilic syndrome has many and varied complications. Their development depends entirely on which organ systems are involved in the disease process. […] The presence of features that are suggestive of myeloproliferative disorder (MPD) and leukocytosis greater than 90,000/ L carry a worse prognosis in hypereosinophilic syndrome.

• #34 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Pulmonary manifestations of HES include asthma-like symptoms, including dyspnea, cough, and wheezing. Abnormal findings such as patchy ground-glass infiltrates and consolidation within the pulmonary parenchyma are observed on chest radiography or computed tomography (CT). […] Gastrointestinal involvement includes colitis or enteritis, eosinophilic gastritis, chronic active hepatitis, focal hepatic lesions, eosinophilic cholangitis, and Budd-Chiari syndrome. These may present as dysphagia, weight loss, abdominal pain, nausea, vomiting, and diarrhea. […] Cardiac manifestations exhibit a higher incidence in FIP1L1-PDGFRA-associated HES. This phenomenon arises from the infiltration of eosinophils into the myocardium, leading to myocardial necrosis due to the release of toxic cationic proteins and enhanced leukocyte recruitment or activation.

• #35 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Pulmonary manifestations of HES include asthma-like symptoms, including dyspnea, cough, and wheezing. Abnormal findings such as patchy ground-glass infiltrates and consolidation within the pulmonary parenchyma are observed on chest radiography or computed tomography (CT). […] Gastrointestinal involvement includes colitis or enteritis, eosinophilic gastritis, chronic active hepatitis, focal hepatic lesions, eosinophilic cholangitis, and Budd-Chiari syndrome. These may present as dysphagia, weight loss, abdominal pain, nausea, vomiting, and diarrhea. […] Cardiac manifestations exhibit a higher incidence in FIP1L1-PDGFRA-associated HES. This phenomenon arises from the infiltration of eosinophils into the myocardium, leading to myocardial necrosis due to the release of toxic cationic proteins and enhanced leukocyte recruitment or activation.

• #36 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  The progression of eosinophil-mediated heart damage comprises 3 main stages, which do not always occur in sequence. The acute necrotic stage, often clinically silent with a normal physical exam, involves myocardial infiltration with eosinophils and lymphocytes, followed by eosinophil degranulation, myocardial necrosis, and the formation of sterile microabscesses. […] In the intermediate phase, thrombus formation occurs due to eosinophil peroxidase generating an acidic compound called hypothiocyanous acid, which diffuses into the endothelial cells, inducing tissue factor expression. […] The fibrotic stage, known as eosinophilic myocardial fibrosis (EMF), occurs secondary to myocardial scarring. It may involve ventricles and the subvalvular apparatus or mitral and tricuspid valves. EMF is responsible for restrictive cardiomyopathy of poor prognosis as well as fatal ventricular arrhythmias secondary to alterations of the cardiac conduction system.

• #37 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  The progression of eosinophil-mediated heart damage comprises 3 main stages, which do not always occur in sequence. The acute necrotic stage, often clinically silent with a normal physical exam, involves myocardial infiltration with eosinophils and lymphocytes, followed by eosinophil degranulation, myocardial necrosis, and the formation of sterile microabscesses. […] In the intermediate phase, thrombus formation occurs due to eosinophil peroxidase generating an acidic compound called hypothiocyanous acid, which diffuses into the endothelial cells, inducing tissue factor expression. […] The fibrotic stage, known as eosinophilic myocardial fibrosis (EMF), occurs secondary to myocardial scarring. It may involve ventricles and the subvalvular apparatus or mitral and tricuspid valves. EMF is responsible for restrictive cardiomyopathy of poor prognosis as well as fatal ventricular arrhythmias secondary to alterations of the cardiac conduction system.

• #38 Hypereosinophilic syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hypereosinophilic_syndrome

  Symptoms Fatigue, breathlessness, cough, muscle pain, fever, and rash. […] Hypereosinophilic syndrome can manifest in many different ways from nonspecific symptoms and fatigue to neurological impairment and endomyocardial fibrosis, which may be fatal. […] Patients can develop a range of nonspecific symptoms, including fever, diarrhea, rash, angioedema, weakness, exhaustion, coughing, and dyspnea. […] The common and non-specific cutaneous manifestations are either erythematous, itchy papules and nodules that resemble eczema, or urticarial and angioedematous lesions. […] Cardiac involvement typically progresses through three phases. Rarely, the early necrotic stage involving the endo-myocardium manifests as acute heart failure. In most cases, however, there are no symptoms. A thrombotic stage ensues after this one, during which thrombi form in the cardiac chambers along the injured endocardium and may separate, resulting in peripheral emboli. Endomyocardial fibrosis causes irreversible restrictive cardiomyopathy in the final stage of fibrosis, and damage to the atrioventricular valves may cause more acute presentations of congestive heart failure.

• #39 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Neurologic manifestations of HES may present as central or peripheral symptoms. Central symptoms include encephalopathy and organic psychosyndrome. Peripheral symptoms account for over 50% of neurologic symptoms of HES and present as peripheral neuropathy, mononeuropathy multiplex, autonomic neuropathy, and polymyositis. […] The true incidence and prevalence of hypereosinophilic syndromes is unknown. A 2010 study utilizing the Surveillance, Epidemiology, and End Results (SEER) database showed an estimated age-adjusted incidence rate between 0.16 and 0.36 per 100,000 and prevalence (calculated as a product of incidence times duration of chronic disease) between 0.36 to 6.3 per 100,000. […] While HES occurs more commonly between the ages of 20 to 50, some pediatric cases are reported. Studies suggest that the frequencies of HES variants are similar between children and adults. Children with primary immunodeficiency more commonly present with secondary HES than adults. In addition, children were noted to have higher median peak absolute eosinophil count, more gastrointestinal complaints, and less pulmonary involvement.

• #40 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Neurologic manifestations of HES may present as central or peripheral symptoms. Central symptoms include encephalopathy and organic psychosyndrome. Peripheral symptoms account for over 50% of neurologic symptoms of HES and present as peripheral neuropathy, mononeuropathy multiplex, autonomic neuropathy, and polymyositis. […] The true incidence and prevalence of hypereosinophilic syndromes is unknown. A 2010 study utilizing the Surveillance, Epidemiology, and End Results (SEER) database showed an estimated age-adjusted incidence rate between 0.16 and 0.36 per 100,000 and prevalence (calculated as a product of incidence times duration of chronic disease) between 0.36 to 6.3 per 100,000. […] While HES occurs more commonly between the ages of 20 to 50, some pediatric cases are reported. Studies suggest that the frequencies of HES variants are similar between children and adults. Children with primary immunodeficiency more commonly present with secondary HES than adults. In addition, children were noted to have higher median peak absolute eosinophil count, more gastrointestinal complaints, and less pulmonary involvement.

• #41 Hypereosinophilic syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hypereosinophilic_syndrome

  Both the peripheral (polyneuropathy) and central (diffuse encephalopathy) nervous systems may be affected by neurological manifestations. Disorientation, memory loss, and altered behavior and cognitive function are the symptoms of diffuse encephalopathy. Symptoms of peripheral neuropathies can include mixed sensory and motor complaints, symmetric or asymmetric sensory alterations, or pure motor deficits. […] When there are no radiological abnormalities, lung involvement can vary from a persistent dry cough and/or bronchial hyperreactivity to restrictive disease with pulmonary infiltrates. […] Abdominal pain, diarrhea, nausea, and vomiting are a few examples of gastrointestinal symptoms. […] If left untreated, HES is progressive and fatal.

• #42 Idiopathic hypereosinophilic syndrome presenting as mononeuritis multiplex. Lessons learned after 18-months of follow-up | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-idiopathic-hypereosinophilic-syndrome-presenting-as-S2173580821000407

  Hypereosinophilic syndrome (HS) is a rare systemic disease included in the group of myeloproliferative disorders. It is diagnosed in patients presenting elevated eosinophil counts below 1500cells/L for over 6 consecutive months and eosinophilic infiltration of tissues and organs after excluding such secondary causes of eosinophilia as drug reactions, infections, immune disorders, or allergies. […] From a neurological viewpoint, idiopathic HS is characterised by involvement of both the central nervous system (venous thrombosis and encephalopathy) and the peripheral nervous system (peripheral neuropathy). […] We present the case of a 49-year-old woman with no relevant medical history who presented mononeuritis multiplex as the initial manifestation of idiopathic HS. She consulted due to one months history of acute pain beginning in the left forearm, and subsequently affecting the right elbow and the anterior aspect of the right thigh. Pain progressed to persistent paraesthesia and weakness in the areas previously mentioned.

• #43 Hypereosinophilic Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/202030-overview

  Hypereosinophilic syndrome (HES) is a myeloproliferative disorder (MPD) characterized by persistent eosinophilia that is associated with damage to multiple organs. […] Hypereosinophilic syndrome carries a variable prognosis. It is a chronic and progressive disorder that is potentially fatal if left untreated. […] The most serious complication of hypereosinophilic syndrome is cardiac involvement, which can lead to myocardial fibrosis, chronic heart failure, and death. […] Patients with HES with the PDGFRA mutation have a very high incidence of cardiac involvement and carry a poor prognosis without therapy. […] Hypereosinophilic syndrome has many and varied complications. Their development depends entirely on which organ systems are involved in the disease process. […] The presence of features that are suggestive of myeloproliferative disorder (MPD) and leukocytosis greater than 90,000/ L carry a worse prognosis in hypereosinophilic syndrome.

• #44

  https://link.springer.com/article/10.1007/s40629-022-00221-w

  The prognosis of HES also depends on the type and extent of organ involvement. With definitive exclusion of the diseases associated with clonal eosinophilia, especially the patients with cardiac involvement have potentially lethal courses due to endomyocarditis, thromboembolic complications, and heart failure. […] For adequate treatment of HES, careful clarification of the qualitative and quantitative organ involvement pattern is essential.

• #45 Hypereosinophilic Syndrome – Ask Hematologist | Understand Hematology

  https://askhematologist.com/hypereosinophilic-syndrome/

  The prognosis of HES depends upon the extent of any organ damage. In very severe cases, HES may be fatal, but there is hope. Survival rates have improved greatly. In 1975, only 12% of HES patients survived three years. Today, more than 80% of HES patients survive five years or more. […] Death usually results from an organ, particularly the heart, dysfunction. Cardiac involvement is not predicted by the degree or duration of eosinophilia. The prognosis varies depending on the response to therapy. Response to imatinib improves the prognosis among patients with the FIP1L1/PDGFRA-associated fusion gene and other responsive gene fusions. Current therapy has improved the prognosis.

• #46 Hypereosinophilic Syndrome – Ask Hematologist | Understand Hematology

  https://askhematologist.com/hypereosinophilic-syndrome/

  The prognosis of HES depends upon the extent of any organ damage. In very severe cases, HES may be fatal, but there is hope. Survival rates have improved greatly. In 1975, only 12% of HES patients survived three years. Today, more than 80% of HES patients survive five years or more. […] Death usually results from an organ, particularly the heart, dysfunction. Cardiac involvement is not predicted by the degree or duration of eosinophilia. The prognosis varies depending on the response to therapy. Response to imatinib improves the prognosis among patients with the FIP1L1/PDGFRA-associated fusion gene and other responsive gene fusions. Current therapy has improved the prognosis.

• #47 Chronic Eosinophilic Leukemia-Not Otherwise Specified (CEL-NOS)
  Idiopathic Hypereosinophilic Syndrome (IHES)

  https://atlasgeneticsoncology.org/haematological/1340/chronic-eosinophilic-leukemia-not-otherwise-specified-(cel-nos)-br-idiopathic-hypereosinophilic-syndrome-(ihes)

  Patients may present with various combinations of symptoms and signs of end-organ damage mediated by eosinophils. […] The common constitutional symptoms that patients experience are fatigue, cough, dyspnea, myalgia, fever, diarrhea, rash and/or rhinitis. […] Progressive heart failure is an example of eosinophil-mediated organ injury which is the major cause of morbidity and mortality in these patients. […] Pulmonary disease affects up to 50% of those patients. […] CNS dysfunction, peripheral neuropathy, GI disorders and skin lesions are also frequent manifestations. […] The incidence of CEL-NOS is not well-defined due to rarity of the disorders and difficulty to distinguish CEL-NOS from idiopathic HES. […] The epidemiology features of idiopathic HES remain undefined. […] In a small series of 10 patients with CEL-NOS, the median survival time was little over 22 months with 50% of patients transformed to acute leukemia. […] However, idiopathic HES is more heterogeneous and the median survival is longer than that of CEL-NOS. […] Features that signify a better prognosis include the absence of cardiac or neurologic involvement, lower eosinophil counts, and steroid-responsiveness.

• #48 Hypereosinophilic Syndrome – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/eosinophilic-disorders/hypereosinophilic-syndrome

  Hypereosinophilic syndrome is a condition characterized by peripheral blood eosinophilia with manifestations of organ system involvement or dysfunction directly related to eosinophilia in the absence of parasitic, allergic, or other secondary causes of eosinophilia. Symptoms can be myriad, depending on which organs are dysfunctional. […] Symptoms are diverse and depend on which organs are dysfunctional. […] Occasionally, patients with very severe eosinophilia (eg, eosinophil counts of 100,000/mcL [ 100 109/L]) develop complications of hyperleukocytosis, such as manifestations of brain or lung hypoxia (eg, encephalopathy, dyspnea, respiratory failure). Other thrombotic manifestations (eg, cardiac mural thrombi) may also occur. […] Death usually results from organ, particularly cardiac, dysfunction. Cardiac involvement is not predicted by the degree or duration of eosinophilia. Prognosis varies depending on response to therapy. Response to imatinib improves the prognosis among patients with the FIP1L1/PDGFRA-associated fusion gene and other responsive gene fusions.

• #49 Eosinophilic Disorders – Blood Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/blood-disorders/white-blood-cell-disorders/eosinophilic-disorders

  Hypereosinophilic syndrome is an uncommon disorder in which the number of eosinophils increases to more than 1,500 cells per microliter of blood (more than 1.5 109 per liter) for more than 6 months without an obvious cause. […] The increased number of eosinophils can damage the heart, lungs, liver, skin, and nervous system. […] Symptoms may include weight loss, fevers, night sweats, fatigue, cough, chest pain, swelling, stomachache, rash, pain, weakness, confusion, and coma. Additional symptoms of this syndrome depend on which organs are damaged. […] The syndrome is suspected when repeated blood tests reveal that the number of eosinophils is persistently increased in people who have these symptoms. […] Without treatment, generally more than 80% of the people who have this syndrome die within 2 years, but with treatment, more than 80% survive. Heart damage is the principal cause of death.

• #50 Hypereosinophilic Syndrome – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/eosinophilic-disorders/hypereosinophilic-syndrome

  Hypereosinophilic syndrome is a condition characterized by peripheral blood eosinophilia with manifestations of organ system involvement or dysfunction directly related to eosinophilia in the absence of parasitic, allergic, or other secondary causes of eosinophilia. Symptoms can be myriad, depending on which organs are dysfunctional. […] Symptoms are diverse and depend on which organs are dysfunctional. […] Occasionally, patients with very severe eosinophilia (eg, eosinophil counts of 100,000/mcL [ 100 109/L]) develop complications of hyperleukocytosis, such as manifestations of brain or lung hypoxia (eg, encephalopathy, dyspnea, respiratory failure). Other thrombotic manifestations (eg, cardiac mural thrombi) may also occur. […] Death usually results from organ, particularly cardiac, dysfunction. Cardiac involvement is not predicted by the degree or duration of eosinophilia. Prognosis varies depending on response to therapy. Response to imatinib improves the prognosis among patients with the FIP1L1/PDGFRA-associated fusion gene and other responsive gene fusions.

• #51 Hypereosinophilic syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hypereosinophilic_syndrome

  Both the peripheral (polyneuropathy) and central (diffuse encephalopathy) nervous systems may be affected by neurological manifestations. Disorientation, memory loss, and altered behavior and cognitive function are the symptoms of diffuse encephalopathy. Symptoms of peripheral neuropathies can include mixed sensory and motor complaints, symmetric or asymmetric sensory alterations, or pure motor deficits. […] When there are no radiological abnormalities, lung involvement can vary from a persistent dry cough and/or bronchial hyperreactivity to restrictive disease with pulmonary infiltrates. […] Abdominal pain, diarrhea, nausea, and vomiting are a few examples of gastrointestinal symptoms. […] If left untreated, HES is progressive and fatal.

• #52 Eosinophilic Disorders – Blood Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/blood-disorders/white-blood-cell-disorders/eosinophilic-disorders

  Hypereosinophilic syndrome is an uncommon disorder in which the number of eosinophils increases to more than 1,500 cells per microliter of blood (more than 1.5 109 per liter) for more than 6 months without an obvious cause. […] The increased number of eosinophils can damage the heart, lungs, liver, skin, and nervous system. […] Symptoms may include weight loss, fevers, night sweats, fatigue, cough, chest pain, swelling, stomachache, rash, pain, weakness, confusion, and coma. Additional symptoms of this syndrome depend on which organs are damaged. […] The syndrome is suspected when repeated blood tests reveal that the number of eosinophils is persistently increased in people who have these symptoms. […] Without treatment, generally more than 80% of the people who have this syndrome die within 2 years, but with treatment, more than 80% survive. Heart damage is the principal cause of death.

• #53 Hypereosinophilic syndrome

  https://dermnetnz.org/topics/hypereosinophilic-syndrome

  Hypereosinophilic syndrome usually presents with fever, weight loss, fatigue, and rash. […] A rash is present in over 50% of patients, but is non-specific in appearance. Most commonly, red swollen itchy nodules (lumps) have been described. It can also resemble urticaria (hives). […] An enlarged liver and spleen is often present indicating liver and spleen involvement. The lungs, kidneys, heart and nervous system can be affected. […] The overall 5-year survival for patients with hypereosinophilic syndrome is 80% and the cause of death is usually heart failure.

• #54 Hypereosinophilic Syndrome: What It Is, Causes, Complications

  https://www.verywellhealth.com/hypereosinophilic-syndrome-7564454

  Hypereosinophilic syndromes affect organs other than the skin. HES in the heart, lungs, stomach, intestines, and other organs can lead to organ dysfunction. Almost half (44%) of people with HES have lung symptoms, and 38% have intestinal issues. Eventually, about 20% develop heart issues. […] Other common symptoms of hypereosinophilic syndromes include weakness and fatigue, cough, trouble breathing, muscle aches and pain, swelling of the deeper layers of the skin, caused by a buildup of fluid, fever, and a runny nose, called rhinitis. […] HES prognosis depends on what’s causing the disease. It cannot be cured, but medications can control most types. How the disease responds to these treatments plays a significant role in how the condition plays out. […] Prognosis depends on what organs the eosinophils are attacking and how much damage they’ve done. If the damage is already severe, it can’t be undone, and HES may be fatal. The most common cause of death in cases of HES is heart failure. Today, more than 80% of HES patients survive five years or more.

• #55 Hypereosinophilic Syndrome (HES): Types, Symptoms, & More

  https://www.health.com/hypereosinophilic-syndrome-8546797

  Symptoms of HES vary depending on the organ system affected but commonly include skin rash, fever, fatigue, muscle aches, weakness, and weight loss. […] Symptoms common with all types of HES include: Skin rash, which may be red, itchy bumps (urticaria/hives) or swollen lumps under the skin (angioedema), Fatigue, Fever, Muscle aches, Weakness, Loss of appetite and/or unintentional weight loss, Cough, Shortness of breath, Memory problems, Confusion. […] Treatment for hypereosinophilic syndrome focuses on decreasing high eosinophil levels in your blood, reducing inflammation to prevent organ damage, and slowing disease progression. […] Untreated HES can cause extensive damage to the heart, lungs, gastrointestinal tract, and other organs, increasing your risk of life-threatening complications.

• #56 Hypereosinophilic syndrome – wikidoc

  https://www.wikidoc.org/index.php/Hypereosinophilic_syndrome

  Hypereosinophilic syndrome (HES) is a type of myeloproliferative disorder characterized by a persistently elevated eosinophil count ( 1500 eosinophils/mm) in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow. […] Patients with hypereosinophilic syndrome may be initially asymptomatic. Early clinical features include fatigue, diarrhea, and rash. […] Prognosis is generally poor, and the 3-year survival rate of patients with hypereosinophilic syndrome is approximately 12%. […] If left untreated, the majority of patients with hypereosinophilic syndrome may progress to develop thromboembolism, acute respiratory failure, and death. […] Common complications of hypereosinophilic syndrome include chronic heart failure, myocardial fibrosis, and death.

• #57 Hypereosinophilic Syndrome

  https://www.aaaai.org/conditions-treatments/related-conditions/hypereosinophilic-syndrome

  Most people have less than 500 eosinophils/microliter in their blood. People with HES usually have more than 1,500 eosinophils/microliter in their blood for 6 months or more, and the cause cannot be identified. […] Symptoms HES can happen at any age, although it is more common in adults. People with HES may suffer from a wide variety of symptoms, depending upon which parts of the body are affected. These symptoms include: Skin rashes such as urticaria or angioedema, Dizziness, Memory loss or confusion, Cough, Shortness of breath, Fatigue, Fever, Mouth sores. […] The prognosis of HES depends upon the extent of any organ damage. In very severe cases, HES may be fatal, but there is hope. Survival rates have improved greatly. In 1975, only 12% of HES patients survived three years. Today, more than 80% of HES patients survive five years or more.

• #58 Hypereosinophilic Syndrome

  https://www.aaaai.org/conditions-treatments/related-conditions/hypereosinophilic-syndrome

  Most people have less than 500 eosinophils/microliter in their blood. People with HES usually have more than 1,500 eosinophils/microliter in their blood for 6 months or more, and the cause cannot be identified. […] Symptoms HES can happen at any age, although it is more common in adults. People with HES may suffer from a wide variety of symptoms, depending upon which parts of the body are affected. These symptoms include: Skin rashes such as urticaria or angioedema, Dizziness, Memory loss or confusion, Cough, Shortness of breath, Fatigue, Fever, Mouth sores. […] The prognosis of HES depends upon the extent of any organ damage. In very severe cases, HES may be fatal, but there is hope. Survival rates have improved greatly. In 1975, only 12% of HES patients survived three years. Today, more than 80% of HES patients survive five years or more.

• #59 Hypereosinophilic Syndrome – Ask Hematologist | Understand Hematology

  https://askhematologist.com/hypereosinophilic-syndrome/

  The prognosis of HES depends upon the extent of any organ damage. In very severe cases, HES may be fatal, but there is hope. Survival rates have improved greatly. In 1975, only 12% of HES patients survived three years. Today, more than 80% of HES patients survive five years or more. […] Death usually results from an organ, particularly the heart, dysfunction. Cardiac involvement is not predicted by the degree or duration of eosinophilia. The prognosis varies depending on the response to therapy. Response to imatinib improves the prognosis among patients with the FIP1L1/PDGFRA-associated fusion gene and other responsive gene fusions. Current therapy has improved the prognosis.

• #60 Hypereosinophilic Syndrome: What It Is, Causes, Diagnosis & Treatment

  https://my.clevelandclinic.org/health/diseases/22541-hypereosinophilic-syndrome

  Hypereosinophilic syndrome happens when eosinophilia accelerates, speeding up eosinophil production and increasing the number of eosinophils. This eosinophil overload can damage your organs, including your heart, lungs, skin and nervous system. Left untreated, hypereosinophilic syndrome can be life-threatening. Fortunately, prompt diagnosis and treatment mean more than 80% of those diagnosed with hypereosinophilic syndrome are alive five years after diagnosis. […] The most common HES symptom is a rash. Hypereosinophilic symptoms correlate (match to) to the area of your body affected by high eosinophil levels. For example, if your heart has an unusually high level of eosinophils, you might have symptoms of congestive heart failure, cardiomyopathy, myocarditis and pericardial effusion. Likewise, if the eosinophils are congregating in your lungs, you might have recurring (repeating) upper respiratory infections, coughing and difficulty breathing. […] Generally speaking, the sooner your HES is diagnosed and treated, the less likely you are to have lasting problems.

• #61 What Is Hypereosinophilic Syndrome?

  https://www.icliniq.com/articles/blood-health/hypereosinophilic-syndrome

  Symptoms of HES are based on the affected area and include: Skin – Eczema, thickening of the skin, hives. Lungs – Cough, shortness of breath, wheezing. Heart – Chest pain, difficulty breathing. Neurologic Disease – Central nervous system dysfunction and peripheral nephropathy. Thrombosis – Formation of blood clots. Eyes – Impaired vision. Gastrointestinal Tract – Loss of appetite and weight, pain, vomiting, diarrhea. […] The earlier HES is diagnosed and treated; the less likely a permanent problem will occur.

• #62 Hypereosinophilic syndrome – wikidoc

  https://www.wikidoc.org/index.php/Hypereosinophilic_syndrome

  Symptoms of hypereosinophilic syndrome may include the following: Shortness of breath, Skin rash, Cough, Diarrhea, Myalgias, Fatigue, Weight-loss. […] Patients with hypereosinophilic syndrome usually have a normal appearance. Physical examination may be remarkable for: Skin rash, Thickening of the skin (lichenification), Eczema (flexural areas), Dermographism, Low-grade fever, Raynaud phenomenon, Wheezing. […] Findings related with poor prognosis in hypereosinophilic syndrome, include: presence of anemia, thrombocytopenia, white blood cell count greater than 100,000 cells/cm3, abnormal marrow and/or basophils, elevated serum levels of vitamin B12, serum tryptase, and abnormal levels of leukocyte alkaline phosphatase.

• #63 Hypereosinophilic Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/202030-overview

  Hypereosinophilic syndrome (HES) is a myeloproliferative disorder (MPD) characterized by persistent eosinophilia that is associated with damage to multiple organs. […] Hypereosinophilic syndrome carries a variable prognosis. It is a chronic and progressive disorder that is potentially fatal if left untreated. […] The most serious complication of hypereosinophilic syndrome is cardiac involvement, which can lead to myocardial fibrosis, chronic heart failure, and death. […] Patients with HES with the PDGFRA mutation have a very high incidence of cardiac involvement and carry a poor prognosis without therapy. […] Hypereosinophilic syndrome has many and varied complications. Their development depends entirely on which organ systems are involved in the disease process. […] The presence of features that are suggestive of myeloproliferative disorder (MPD) and leukocytosis greater than 90,000/ L carry a worse prognosis in hypereosinophilic syndrome.

• #64 Hypereosinophilic Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/202030-overview

  Hypereosinophilic syndrome (HES) is a myeloproliferative disorder (MPD) characterized by persistent eosinophilia that is associated with damage to multiple organs. […] Hypereosinophilic syndrome carries a variable prognosis. It is a chronic and progressive disorder that is potentially fatal if left untreated. […] The most serious complication of hypereosinophilic syndrome is cardiac involvement, which can lead to myocardial fibrosis, chronic heart failure, and death. […] Patients with HES with the PDGFRA mutation have a very high incidence of cardiac involvement and carry a poor prognosis without therapy. […] Hypereosinophilic syndrome has many and varied complications. Their development depends entirely on which organ systems are involved in the disease process. […] The presence of features that are suggestive of myeloproliferative disorder (MPD) and leukocytosis greater than 90,000/ L carry a worse prognosis in hypereosinophilic syndrome.

• #65 Hypereosinophilic Syndrome – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/eosinophilic-disorders/hypereosinophilic-syndrome

  Hypereosinophilic syndrome is a condition characterized by peripheral blood eosinophilia with manifestations of organ system involvement or dysfunction directly related to eosinophilia in the absence of parasitic, allergic, or other secondary causes of eosinophilia. Symptoms can be myriad, depending on which organs are dysfunctional. […] Symptoms are diverse and depend on which organs are dysfunctional. […] Occasionally, patients with very severe eosinophilia (eg, eosinophil counts of 100,000/mcL [ 100 109/L]) develop complications of hyperleukocytosis, such as manifestations of brain or lung hypoxia (eg, encephalopathy, dyspnea, respiratory failure). Other thrombotic manifestations (eg, cardiac mural thrombi) may also occur. […] Death usually results from organ, particularly cardiac, dysfunction. Cardiac involvement is not predicted by the degree or duration of eosinophilia. Prognosis varies depending on response to therapy. Response to imatinib improves the prognosis among patients with the FIP1L1/PDGFRA-associated fusion gene and other responsive gene fusions.

• #66 Hypereosinophilic Syndrome – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/eosinophilic-disorders/hypereosinophilic-syndrome

  Hypereosinophilic syndrome is a condition characterized by peripheral blood eosinophilia with manifestations of organ system involvement or dysfunction directly related to eosinophilia in the absence of parasitic, allergic, or other secondary causes of eosinophilia. Symptoms can be myriad, depending on which organs are dysfunctional. […] Symptoms are diverse and depend on which organs are dysfunctional. […] Occasionally, patients with very severe eosinophilia (eg, eosinophil counts of 100,000/mcL [ 100 109/L]) develop complications of hyperleukocytosis, such as manifestations of brain or lung hypoxia (eg, encephalopathy, dyspnea, respiratory failure). Other thrombotic manifestations (eg, cardiac mural thrombi) may also occur. […] Death usually results from organ, particularly cardiac, dysfunction. Cardiac involvement is not predicted by the degree or duration of eosinophilia. Prognosis varies depending on response to therapy. Response to imatinib improves the prognosis among patients with the FIP1L1/PDGFRA-associated fusion gene and other responsive gene fusions.

• #67 Hypereosinophilic Syndrome – Ask Hematologist | Understand Hematology

  https://askhematologist.com/hypereosinophilic-syndrome/

  The signs and symptoms of HES can vary significantly depending on which part(s) of the body are affected. Frequent symptoms listed by body system include: Skin rashes, itching, and edema. Lung asthma, cough, difficulty breathing, recurrent upper respiratory infections, and pleural effusion. Gastrointestinal abdominal pain, vomiting, and diarrhea. Musculoskeletal arthritis, muscle inflammation, muscle aches, and joint pain. Nervous system vertigo, paresthesia, speech impairment, and visual disturbances. Heart congestive heart failure, cardiomyopathy, pericardial effusion, and myocarditis. Blood deep venous thrombosis, and anemia. […] Affected people can also experience a variety of non-specific symptoms such as fever, weight loss, night sweats, and fatigue. […] Occasionally, patients with very severe eosinophilia (eg, eosinophil counts of 100,000/mcL [ 100 109/L]) develop complications of hyperleukocytosis, such as manifestations of brain or lung hypoxia (eg, encephalopathy, dyspnea, respiratory failure). Other thrombotic manifestations (eg, cardiac mural thrombi) may also occur.

• #68 Idiopathic Hypereosinophilic Syndrome with Multiple Organ Involvement

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7983584/

  Pulmonary involvement is common in HES and may result from eosinophilic infiltration of the lung with subsequent fibrosis, heart failure, or pulmonary emboli. Eosinophilic gastritis, enteritis, and/or colitis may occur secondary to HES and cause weight loss, abdominal pain, vomiting, and/or severe diarrhea. […] Patients with idiopathic HES should be treated in the first instance with corticosteroids. Patients requiring emergency treatment for severe or life-threatening eosinophilia should receive high-dose corticosteroids. Patients with idiopathic HES who do not respond adequately to corticosteroids, who require prolonged corticosteroid therapy, or who are intolerant of corticosteroids should be considered for a short trial of imatinib, immunomodulatory agents, myelosuppressive therapy, or monoclonal antibody therapy with mepolizumab. […] The thromboembolic disease associated with HES is particularly difficult to control.

• #69 Hypereosinophilic syndrome presenting as coagulopathy | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-022-00666-2

  Hypereosinophilic syndrome (HES) is an extremely uncommon group of disorders. It rarely presents with coagulopathy without cardiac involvement. […] HES typically presents with symptoms of weakness and fatigue, cough, dyspnea, myalgia, angioedema, rash, or fever. […] The most common organ systems involved with presentation of HES are dermatologic (69%), pulmonary (44%), and gastrointestinal (38%). […] Thromboembolic events are thought to occur in 25% of patients with cardiac involvement of HES, which has previously been attributed to endocardial damage leading to mural thrombi. […] In patients with an unexplained coagulopathy and eosinophilia, eosinophilic disorders such as HES should be considered. […] Our patient highlights the importance of having an increased index of suspicion for HES in patients presenting with eosinophilia and thrombosis. Rapid diagnosis of HES and utilization of steroid-sparing agents such as anti-IL-5 therapies could help prevent further end-organ damage, treat an underlying mechanism for associated coagulopathy, and avoid long-term side effects of corticosteroid use.

• #70 A Hypereosinophilic Syndrome Presenting as Eosinophilic Colitis

  https://www.e-ce.org/journal/view.php?number=6442

  The patient was finally diagnosed with HES with involvement of the colon, stomach, duodenum, liver, and lung. Because hypereosinophilia was present for 3 months before his HES diagnosis, hypereosinophilic period was shorter than the diagnostic criteria for HES. However, oral corticosteroid therapy (prednisolone, 60 mg/day) was started for HES with severe symptoms caused by multiple organ involvement. Three days later, symptoms such as abdominal pain, bloody diarrhea, and cough were improved dramatically. […] Because HES can involve multiple organs and have fatal course, we think eosinophil-lowering therapy should be started as soon as possible to prevent aggressive disease progression and organ dysfunction.

• #71 Hypereosinophilic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK599558/

  Neurologic manifestations of HES may present as central or peripheral symptoms. Central symptoms include encephalopathy and organic psychosyndrome. Peripheral symptoms account for over 50% of neurologic symptoms of HES and present as peripheral neuropathy, mononeuropathy multiplex, autonomic neuropathy, and polymyositis. […] The true incidence and prevalence of hypereosinophilic syndromes is unknown. A 2010 study utilizing the Surveillance, Epidemiology, and End Results (SEER) database showed an estimated age-adjusted incidence rate between 0.16 and 0.36 per 100,000 and prevalence (calculated as a product of incidence times duration of chronic disease) between 0.36 to 6.3 per 100,000. […] While HES occurs more commonly between the ages of 20 to 50, some pediatric cases are reported. Studies suggest that the frequencies of HES variants are similar between children and adults. Children with primary immunodeficiency more commonly present with secondary HES than adults. In addition, children were noted to have higher median peak absolute eosinophil count, more gastrointestinal complaints, and less pulmonary involvement.

• #72 Hypereosinophilic syndrome (Idiopathic hypereosinophilic syndrome) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/hypereosinophilic-syndrome-idiopathic-hypereosinophilic-syndrome/

  The M-HES can present with skin lesions in addition to fever, weight loss, fatigue, enlarged liver and spleen. […] Mucosal ulcerations have been reported as a distinct feature of M-HES. Mucosal lesions are typically painful and may occur on oropharynx, lips, tongue, buccal and gingival surfaces, conjunctiva or genital mucosa as erosions, ulcerations, or aphthous-like lesions. […] A subset of HES known as undefined HES has been described, which manifests as cyclic or episodic angioedema with urticaria and fever along with marked eosinophilia and increase in body weight. […] Considering the great variability in pathogenesis and clinical presentations, the long-term course and prognosis is variable. This may range from minimal impairment requiring no treatment, to severe disease leading to disability. […] Poor outcomes are related to severe organ damage, particularly heart failure and malignant transformations. M-HES can transform to acute myelogenous leukemia and L-HES to T-cell lymphomas.

• #73 Hypereosinophilic Syndrome and T-Cell Lymphoma: Which Comes First? | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-hypereosinophilic-syndrome-t-cell-lymphoma-articulo-S0001731023005902

  Hypereosinophilic syndrome (HES) is defined by a concentration of eosinophils in peripheral blood greater than 1500cells/L, accompanied by organ damage or dysfunction not attributable to other causes. […] Close follow-up of these patients is essential, as 10-20% may progress to cutaneous T-cell lymphoma (CTCL) with a poor prognosis. […] A 67-year-old man attended the clinic with symptoms of 3 months standing of polyarthralgia, multiple migratory bilateral pulmonary infiltrates, generalized highly itchy maculopapular skin rash, and constitutional syndrome with asthenia, anorexia, and weight loss. […] Eosinophilia has an important prognostic role in CTCL, with median survival 3 years lower in patients with eosinophil levels above 700/L. Some authors have considered eosinophilia on diagnosis as the only prognostic variable associated with disease progression and disease-related death.

• #74 Hypereosinophilic Syndrome and T-Cell Lymphoma: Which Comes First? | Actas Dermo-Sifiliográficas

  https://www.actasdermo.org/es-hypereosinophilic-syndrome-t-cell-lymphoma-articulo-S0001731023005902

  Hypereosinophilic syndrome (HES) is defined by a concentration of eosinophils in peripheral blood greater than 1500cells/L, accompanied by organ damage or dysfunction not attributable to other causes. […] Close follow-up of these patients is essential, as 10-20% may progress to cutaneous T-cell lymphoma (CTCL) with a poor prognosis. […] A 67-year-old man attended the clinic with symptoms of 3 months standing of polyarthralgia, multiple migratory bilateral pulmonary infiltrates, generalized highly itchy maculopapular skin rash, and constitutional syndrome with asthenia, anorexia, and weight loss. […] Eosinophilia has an important prognostic role in CTCL, with median survival 3 years lower in patients with eosinophil levels above 700/L. Some authors have considered eosinophilia on diagnosis as the only prognostic variable associated with disease progression and disease-related death.

• #75 Chronic Eosinophilic Leukemia-Not Otherwise Specified (CEL-NOS)
  Idiopathic Hypereosinophilic Syndrome (IHES)

  https://atlasgeneticsoncology.org/haematological/1340/chronic-eosinophilic-leukemia-not-otherwise-specified-(cel-nos)-br-idiopathic-hypereosinophilic-syndrome-(ihes)

  Patients may present with various combinations of symptoms and signs of end-organ damage mediated by eosinophils. […] The common constitutional symptoms that patients experience are fatigue, cough, dyspnea, myalgia, fever, diarrhea, rash and/or rhinitis. […] Progressive heart failure is an example of eosinophil-mediated organ injury which is the major cause of morbidity and mortality in these patients. […] Pulmonary disease affects up to 50% of those patients. […] CNS dysfunction, peripheral neuropathy, GI disorders and skin lesions are also frequent manifestations. […] The incidence of CEL-NOS is not well-defined due to rarity of the disorders and difficulty to distinguish CEL-NOS from idiopathic HES. […] The epidemiology features of idiopathic HES remain undefined. […] In a small series of 10 patients with CEL-NOS, the median survival time was little over 22 months with 50% of patients transformed to acute leukemia. […] However, idiopathic HES is more heterogeneous and the median survival is longer than that of CEL-NOS. […] Features that signify a better prognosis include the absence of cardiac or neurologic involvement, lower eosinophil counts, and steroid-responsiveness.

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