Materiały źródłowe

• #1 Approach to the patient with suspected hypereosinophilic syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9821533/

  Hypereosinophilic syndromes (HES) are a heterogenous group of rare disorders with clinical manifestations ranging from fatigue to life-threatening endomyocardial fibrosis and thromboembolic events. […] Corticosteroids remain the mainstay of initial therapy in the setting of acute, life-threatening PDGFR mutation-negative HES. […] This review provides a case-based discussion of the differential diagnosis of HES, including the classification by clinical HES subtype. Treatment options are reviewed, including novel eosinophil-targeted agents recently approved for the treatment of HES and/or other eosinophil-associated disorders. […] The presence and severity of clinical manifestations should be assessed as this will affect both the nature and urgency of therapeutic intervention. For example, careful monitoring without therapy may be appropriate for asymptomatic HE without evidence of end-organ involvement (hypereosinophilia of undetermined significance), whereas urgent intervention is needed in the context of myocarditis or thromboembolism.

• #2 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. […] Diagnostic criteria include eosinophil count of 1,500/mm3 or higher, presence of organ involvement and exclusion of other causes of eosinophilia for at least 6 months. […] HES may occur with multiple system and organ involvement and findings. In the differential diagnosis of patients presenting with heart failure, pulmonary involvement and eosinophilia, HES must definitely be considered. […] Treatment should begin in HES, which includes organ damage by definition. The main treatment is corticosteroids; it is usually started at a dose of 1 mg/kg, after 1 – 2 weeks of use at this dose, it is gradually reduced over a period of 2 – 3 months. […] Physicians should definitely consider HES in the differential diagnosis of patients presenting with heart failure, pulmonary involvement and eosinophilia. […] As in our case, although other comorbidities, such as COPD, may prevent a correct diagnosis of HES with pulmonary involvement, early diagnosis usually provides the most appropriate treatment.

• #3 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. […] Treatment may include corticosteroids and sometimes imatinib but depends on the specific subtype of hypereosinophilic syndrome. […] Diagnosis involves excluding other causes of eosinophilia plus bone marrow and cytogenetic tests. […] Evaluation for organ damage should include blood chemistry tests (including liver enzymes, creatine kinase, renal function, and troponin); ECG; echocardiography; pulmonary function tests; and CT of the chest, abdomen, and pelvis. […] Give corticosteroids for severe eosinophilia and/or organ damage. Tyrosine kinase inhibitors such as low-dose imatinib may be of benefit in subtypes associated with distinct chromosomal abnormalities.

• #4 A Hypereosinophilic Syndrome Presenting as Eosinophilic Colitis

  https://www.e-ce.org/journal/view.php?doi=10.5946/ce.2012.45.4.444

  Hypereosinophilic syndrome (HES) has three defining features: marked hypereosinophilia for at least 6 months, no confirmed etiology for the eosinophilia, and eosinophilia-related symptoms or organ dysfunction. […] Although the diagnostic criteria for HES should include hypereosinophilic period for at least 6 months, early initiation of therapy may be recommended in HES patients with symptoms. […] The primary goal of treatment is to reduce the eosinophilia to 1,500/mm3 and to prevent further damage to major organs. […] Corticosteroid is effective for reducing the eosinophil count, and antineoplastics are useful for slowing eosinophil production. […] Patients who are negative for FIP1L1/PDGFRA fusion should receive a single daily dose of prednisone (60 mg or 1 mg/kg orally) for 1 to 2 weeks, whereas FIP1L1/PDGFRA fusion-positive patients should receive a tyrosine kinase inhibitor (imatinib mesylate) as a first-line therapy.

• #5 Approach to the patient with suspected hypereosinophilic syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9821533/

  Prednisone remains the mainstay of therapy in the acute setting for severe and/or life-threatening manifestations of HES. If the eosinophilia does not dramatically decrease within 24 to 48 hours, additional therapy should be considered depending on the suspected clinical subtype (ie, imatinib for patients with clinical findings suggestive of a myeloid neoplasm, cyclophosphamide for patients with manifestations suggestive of eosinophilic granulomatosis with polyangiitis). […] Most patients with symptomatic HES respond rapidly to corticosteroid therapy, although toxicity and resistance limit the utility of this therapy in the long term. […] Despite the differences in definitions, the general approach to HE is very similar between World Health Organization and the consensus group. […] Comprehensive clinical evaluation is necessary both to assess end-organ manifestations and determine the most likely etiology and/or clinical subtype of HES, as this information has important therapeutic and prognostic implications.

• #6 Hypereosinophilic syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hypereosinophilic-syndrome/diagnosis-treatment/drc-20352856

  Our caring team of Mayo Clinic experts can help you with your hypereosinophilic syndrome-related health concerns […] Treatment for hypereosinophilic syndrome is aimed at reducing your eosinophil count to prevent tissue damage, especially to your heart. Specific treatment depends on your symptoms, the severity of your condition and the cause of your HES. […] If you have no symptoms and your eosinophil count is low enough, you might require no treatment other than close monitoring for any changes related to HES. […] Systemic corticosteroids, such as prednisone, are the first line treatment. Other treatment options include: Hydroxyurea (Droxia, Hydrea, Siklos), Imatinib (Gleevec), Vincristine. […] Because HES can increase your risk of blood clots, you might also be prescribed blood-thinning medications such as warfarin (Coumadin). […] If nothing else has worked, your doctor might suggest a stem cell or bone marrow transplant.

• #7 Hypereosinophilic syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/hypereosinophilic-syndrome?content_id=CON-20306102

  Treatment for hypereosinophilic syndrome is aimed at reducing your eosinophil count to prevent tissue damage, especially to your heart. Specific treatment depends on your symptoms, the severity of your condition and the cause of your HES. […] Systemic corticosteroids, such as prednisone, are the first line treatment. Other treatment options include: Hydroxyurea (Droxia, Hydrea, Siklos), Imatinib (Gleevec), Vincristine. […] Because HES can increase your risk of blood clots, you might also be prescribed blood-thinning medications such as warfarin (Coumadin). […] If nothing else has worked, your doctor might suggest a stem cell or bone marrow transplant.

• #8 A Hypereosinophilic Syndrome Presenting as Eosinophilic Colitis

  https://www.e-ce.org/journal/view.php?doi=10.5946/ce.2012.45.4.444

  When the eosinophil count is decreased to 1,500/L, the symptoms are usually relieved. […] The steroid is gradually tapered to an alternate-day schedule at the lowest dose, which is a median dose of prednisone (10 mg/day). […] For cases in which steroid therapy is ineffective, alternative approaches must be considered; these include hydroxyurea, interferon-alpha, anti-interleukin-5, and anti-CD52. […] Surgery should be reserved for patients with significant bleeding, perforation, and GI obstruction and for patients who are refractory to medical treatment. […] 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.

• #9 Hypereosinophilic syndrome – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/hypereosinophilic-syndrome/

  Hypereosinophilic Syndrome (HES) is a rare and complex condition characterized by an overproduction of eosinophils, a type of white blood cell, leading to potential organ damage. Understanding and managing HES requires a comprehensive approach, including standard treatments like corticosteroids, advanced therapies for resistant cases, and ongoing monitoring to prevent complications. […] The primary goal in treating HES is to reduce the high eosinophil levels in the blood and tissues. Corticosteroids are the first-line therapy for most forms of HES, especially in cases without the FIP1L1/PDGFRA mutation. […] For patients who do not respond to corticosteroids, second-line therapies such as interferon alpha and hydroxyurea are recommended. In cases with the FIP1L1/PDGFRA mutation, imatinib, a tyrosine kinase inhibitor, is the drug of choice and has shown high efficacy. Additionally, mepolizumab, a monoclonal antibody targeting interleukin-5, has been effective in reducing disease flares in patients without the FIP1L1/PDGFRA mutation.

• #10 Haute Autorité de Santé – NUCALA (mépolizumab) – Syndrome hyperéosinophilique

  https://www.has-sante.fr/jcms/p_3341449/en/nucala-mepolizumab-syndrome-hypereosinophilique

  Favourable opinion for reimbursement as an add-on treatment for adult patients with inadequately controlled lymphocytic or idiopathic hypereosinophilic syndrome. […] Therapeutic improvement in the management of inadequately controlled lymphocytic or idiopathic hypereosinophilic syndrome. […] The main objectives of treatment are to reduce symptoms and control eosinophilia. It is also essential to prevent any adverse effects of the treatments used in order to ensure control of the disease and prevent long-term complications. […] The therapeutic management should be adapted based on the severity of the disease and the type of HES: […] as first-line treatment: corticosteroids are generally administered at moderate or high doses (0.5 to 1 mg/kg of prednisone equivalent); corticosteroids usually enable normalisation of eosinophil levels and an improvement in clinical signs, but it is common for the disease to reappear on gradual dose tapering;

• #11 Haute Autorité de Santé – NUCALA (mépolizumab) – Syndrome hyperéosinophilique

  https://www.has-sante.fr/jcms/p_3341449/en/nucala-mepolizumab-syndrome-hypereosinophilique

  as second-line treatment: immunomodulatory therapies, such as PEGylated interferon alfa or hydroxyurea, are proposed as corticosteroid-sparing treatments; these are used off-label. […] NUCALA (mepolizumab) is a first-line treatment in patients with lymphocytic or idiopathic hypereosinophilic syndrome inadequately controlled by corticosteroids and/or immunomodulators. […] Regular follow-up of patients within one of these centres is essential to ensure the efficacy of treatment and monitor its safety. […] NUCALA (mepolizumab) provides a minor clinical added value (CAV IV) in the care pathway for inadequately controlled lymphocytic or idiopathic hypereosinophilic syndrome.

• #12

  https://journals.lww.com/ajnonline/fulltext/2021/03000/new_treatment_for_hypereosinophilic_syndrome.18.aspx

  Mepolizumab (Nucala) has been approved to treat hypereosinophilic syndrome. […] Nurses should read the package insert for directions on how to reconstitute and administer the drug subcutaneously. Patient education will be needed regarding use of the autoinjector.

• #13 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. […] In patients with an unexplained coagulopathy and eosinophilia, eosinophilic disorders such as HES should be considered. Corticosteroid-sparing agents, such as benralizumab show promise for successfully treating these patients. […] Treatment of HES patients with thrombi have previously include corticosteroids, anticoagulants, and antiplatelet agents, which have many risks when used long term. Recently, anti-interleukin-5 (anti-IL-5) and anti-IL-5-receptor antibody therapies have shown promise as steroid-sparing treatment for HES. […] 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.

• #14 Hypereosinophilic syndrome (HES) | Healthengine Blog

  https://healthinfo.healthengine.com.au/hypereosinophilic-syndrome-hes

  Treatment is directed toward minimising organ damage and suppressing eosinophilia. […] First line treatment is the use of corticosteroids. […] Anticoagulant therapy should also be administered as thrombosis and embolous are common complications. […] Symptomatic therapies to treat secondary problems such as congestive heart failure should be treated appropriately. […] Antihistamines may be used to control and skin reactions. […] Surgical therapy may be needed in severe cases such as heart valve replacement or splenectomy (if there is severe abdominal discomfort).

• #15 Hypereosinophilic syndrome – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/hypereosinophilic-syndrome/

  In life-threatening situations, high-dose corticosteroid therapy should be initiated immediately. If symptoms do not improve, additional agents may be required to rapidly lower eosinophil counts. Supportive therapies, including surgery and medication, may be necessary for cardiac manifestations such as infiltrative cardiomyopathy and heart failure. […] Patients with HES require regular monitoring to ensure that eosinophilia is controlled and to detect any new or worsening organ involvement. Asymptomatic patients are typically monitored with serum troponin measurements and echocardiography at regular intervals. […] Living with HES requires ongoing management and monitoring. Patients must work closely with their healthcare providers to manage symptoms and prevent complications. The disease’s progression and the patient’s quality of life depend on how well the condition is controlled through medication and lifestyle adjustments. […] The future outlook for patients with HES is promising, thanks to ongoing research and the development of new therapeutic strategies. Understanding the molecular pathogenesis of HES is key to developing targeted therapies that can further improve patient outcomes.

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

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

  Healthcare providers focus on reducing the high eosinophil levels in your blood and tissues. Standard treatment includes corticosteroids and chemotherapy drugs. […] Healthcare providers usually treat hypereosinophilic syndrome with corticosteroids and chemotherapy. Both treatments have side effects. Talk to your healthcare provider about treatment side effects and what you can do to lessen their impact. […] If you’ve been diagnosed with HES, you should contact your healthcare provider if your symptoms persist or get worse. […] Hypereosinophilic syndrome affects several organs, including your heart. If HES affects your heart, you should go to the emergency room any time you have symptoms such as chest pain, heartburn, nausea and sweating. […] The good news is, once your HES is diagnosed, there are successful treatments for it. And while it might take a few months for your treatment to work, try to be patient and be sure to talk to your healthcare provider if you’re having trouble coping with the challenges of a long-term illness. They’ll have suggestions for programs and resources for you.

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