Materiały źródłowe

• #1 Thrombocytosis and Essential Thrombocythemia | Oncohema Key

  https://oncohemakey.com/thrombocytosis-and-essential-thrombocythemia/

  Thrombocytosis and Essential Thrombocythemia George M. Rodgers Robert T. Means, Jr. The normal reference interval for platelet counts in adults approximates 150,000 to 450,000/l; patients with platelet counts higher than the upper limit of normal have thrombocytosis, which can be broadly classified as reactive (secondary), essential (clonal or primary), or inherited. Routine screening of healthy individuals has identified persons with asymptomatic thrombocytosis; some of these people will have essential thrombocythemia (ET). In contrast, hospitalized patients with elevated platelet counts usually have reactive thrombocytosis (RT). At least 90% of all patients with thrombocytosis have RT due to an underlying clinical disorder. It is important to distinguish ET from RT or inherited thrombocytosis because patients with the former disorder may be at risk of ischemic or bleeding complications, or evolution to myelofibrosis or acute leukemia, whereas patients with the latter disorders usually require only treatment of their underlying medical condition, with minimal or no treatment in the case of inherited thrombocytosis.

• #2 Essential Thrombocytosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK539709/

  Essential thrombocytosis is the most common type of myeloproliferative neoplasm. Reports are that 1.0 to 2.5 individuals per 100000 have essential thrombocytosis yearly. The prevalence was reported to be 38 to 57 per 100000 between 2008 and 2010 and mostly occurred in females. The incidence of essential thrombocytosis increases with age, with most patients presenting between the ages of 50 and 60. […] According to the World Health Organization, essential thrombocytosis is a disease that occurs when the platelet count is more than 450000 with the presence of Janus kinase 2 (JAK2), Calreticulin (CALR) or myeloproliferative leukemia virus oncogene (MPL) mutation, lacking clonal or reactive causes.

• #3 Essential Thrombocytosis: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/206697-overview

  In the United States, clinicians diagnose approximately 6000 cases of essential thrombocytosis each year. Some researchers speculate that the incidence rate may be several times higher. A study from southeastern Minnesota reported an incidence of 2.38 cases per 100,000 population per year. […] Essential thrombocytosis is more frequent in older patients. The median age at diagnosis is 60 years, and perhaps up to 20% of patients are younger than 40 years. The disease is rare in children. […] In older patients with essential thrombocytosis, the frequency is similar in both sexes. In younger patients, however, essential thrombocytosis occurs more often in women than in men. […] In the US, a higher incidence has been noted in Blacks. Lower incidences have been seen in Hispanics and non-Hispanic Whites and Asians/ Pacific Islanders.

• #4 Essential thrombocytosis overview – wikidoc

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

  The incidence of essential thrombocytosis is approximately 0.6-2.5 cases per 100,000 individuals worldwide annually. […] The prevalence of essential thrombocytosis is approximately 30 per 100,000 individuals worldwide. […] The incidence of essential thrombocytosis increases with age; the median age at diagnosis is 65-70 years. Patients of all age groups may develop essential thrombocytosis. However, essential thrombocytosis commonly affects individuals older than 60 years of age. […] Females are more commonly affected with essential thrombocytosis than males. […] The female to male ratio is approximately 2 to 1.

• #5 Thrombocytosis: Symptoms, Causes, and Treatment | Doctor

  https://patient.info/doctor/thrombocytosis

  How common is thrombocytosis? (epidemiology) […] Essential thrombocytosis is the most common type of myeloproliferative neoplasm. […] The prevalence has been reported to be 38 to 57 per 100,000, with females more often affected. […] The incidence of essential thrombocytosis increases with age, with most patients presenting between 50 and 60 years. […] The incidence is highest during the first three months of life, and preterm infants are more prone than term infants.

• #6 Essential thrombocythemia | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-2-3

  The reported annual incidence rates for ET range from 0.59 to 2.53/100,000 (9/100,000) inhabitants. A population based survey in the city of Gteborg, Sweden, adjusted to standard population reported an incidence rate of 1.55/100,000, just under the incidence rate evaluated in the same conditions for PV (1.97/100,000). The prevalence is around 30/100,000 inhabitants. […] The diagnosis of ET is more frequently established today than in the past, the most likely explanation being a wider use of automatic count in routine examination leading to the diagnosis of more non symptomatic ET patients. […] Since the diagnosis of ET is frequently recognized early in life and the incidence of the disease is around two times higher in females compared to males, the occurrence of ET associated pregnancies is common.

• #7 Essential thrombocytosis epidemiology and demographics – wikidoc

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

  The incidence of essential thrombocytosis is approximately 0.6-2.5 cases per 100,000 individuals worldwide annually. […] The prevalence of essential thrombocytosis is about 30 for every 100,000 people worldwide. […] The incidence of essential thrombocytosis increases with age; the median age at diagnosis is 65-70 years. […] Patients of all age groups may develop essential thrombocytosis. However it commonly affects individuals older than 60 years of age. […] Females are more commonly affected with essential thrombocytosis than males. […] The female to male ratio is approximately 2 to 1. […] The annual incidence of essential thrombocytosis is estimated to be 0.6-2.5 cases per 100,000 individuals worldwide. […] Worldwide, the prevalence of essential thrombocytosis is approximately 30 for every 100,000 individuals worldwide.

• #8 Thrombocytosis and Essential Thrombocythemia | Oncohema Key

  https://oncohemakey.com/thrombocytosis-and-essential-thrombocythemia/

  A retrospective Turkish survey of the incidence and etiology of thrombocytosis over a 5-year period demonstrated that of 124,340 patients who had platelet counts determined, 2,000 patients (1.6%) had at least one platelet count 500,000/ml. RT was identified in 96.7% of these 2,000 patients, with infection being the most common cause of RT. Another survey found that tissue injury (surgery) was the most common cause of RT. Increased thrombopoiesis seen in infectious, inflammatory, or malignant disorders results from cytokines and other acute-phase response mediators active in these circumstances. […] Evolving diagnostic criteria make it difficult to characterize the incidence and prevalence of ET. In studies published between 2001 and 2008, an incidence of 1.5/100,000 was reported. Prevalence based on insurance claims data in the northeastern United States has been reported to be 24 cases/100,000 population. However, these reports antedate the 2008 revision of the WHO diagnostic criteria for ET, which includes a lower platelet threshold and could potentially alter the reported prevalence and incidence. The median age at diagnosis is in the mid-50s in large reported series, and females represent 60% to 70% of cases. Cases have been reported in children but are uncommon. First-degree relatives of ET patients have an increased risk of ET. Risk for developing ET appears to be associated with environmental factors linked to other myeloid neoplasms, such as radon and gamma irradiation. Hairdressers, electricians, and farmers appear to be at increased risk to develop ET compared to other occupations, although it should be remembered that these risks are still quite low.

• #9 Essential_thrombocytosis

  https://www.bionity.com/en/encyclopedia/Essential_thrombocytosis.html

  Essential thrombocytosis is diagnosed at a rate of about 2 to 3 per 100,000 individuals annually. […] The disease usually affects middle aged to elderly individuals, with an average age at diagnosis of 50-60 years, although it can affect children and young adults as well.

• #10 Essential thrombocytosis epidemiology and demographics – wikidoc

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

  Patients of all age groups may develop essential thrombocytosis. However it commonly affects individuals older than 60 years of age. […] The median age at onset is 65-70 years. […] Women in their third decade of life are more commonly affected with essential thrombocytosis than men at same age. The female to male ratio is approximately 2:1.

• #11 Orphanet: Essential thrombocythemia

  https://www.orpha.net/en/disease/detail/3318

  Prevalence is estimated at 1/4,200 in the US and is reported at 1/3,333 in Sweden. […] The female to male ratio is about 2:1.

• #12

  https://link.springer.com/article/10.1007/s00277-021-04485-0

  Secondary thrombocytosis is a frequent secondary finding in childhood infection and inflammation. […] Current literature has highlighted differences in the epidemiology and molecular pathogenesis of childhood thrombocytosis as compared to adults. […] A recent review described annual incidences of essential thrombocythemia (ET), one of the Philadelphia-negative myeloproliferative neoplasms (MPNs), ranging between 0.004 and 0.11 per 100,000 in children aged 0-16 years, while the meta-analysis in adults showed a pooled annual incidence rate of 1.03 per 100,000. […] Thus, in a group of 89 children with clinically diagnosed ET, Randi et al. found approximately 75% to be triple-negative and to display non-clonal disease. […] Due to heterogeneity of adult and childhood ET, it has previously been emphasized that specific diagnostic criteria for MPNs in children are necessary.

• #13 Essential Thrombocythemia Incidentally Discovered in 5-year-old Patient: A Case Report

  https://pubs.sciepub.com/ajmcr/11/3/4/index.html

  Essential thrombocythemia (ET) is considered a classic BCR-ABL1-negative chronic myeloproliferative condition (MPD) thats characterized by thrombocytosis, bleeding, and/or thrombosis. […] In children under the age of 14, the annual incidence of ET is estimated to be 1 per 10 million, approximately 60 times lower than older ages. […] The clinical entity of essential thrombocythemia is rare in middle-aged adults, and it’s far rarer in pediatrics with an estimated prevalence of 1 in 10 million. […] Limited data is available on incidence of ET in children, ET is estimated to be diagnosed in 1/1,000,000 children. […] ET is 60 times more common in adults than in pediatric patients. […] The incidence of ET is 1-2.5 cases per 100,000 per year. […] Although thrombocytosis is generally a common finding in pediatrics, the primary (even essential) cause is extremely rare. […] In children, the two most common causes of increased platelet count are infections and iron deficiency anemia (IDA). […] To make the diagnosis ET, other causes of secondary (also known as reactive) thrombocytosis must be excluded. […] The Diagnosis of ET is made using the 2016 WHO criteria.

• #14 Essential thrombocythemia – Wikipedia

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

  The incidence of ET is 0.6-2.5/100,000 per year, the median age at onset is 65-70 years and it is more frequent in females than in males. […] The incidence in children is 0.09/100,000 per year.

• #15 The epidemiology and clinical characteristics of myeloproliferative neoplasms in Malaysia | Experimental Hematology & Oncology | Full Text

  https://ehoonline.biomedcentral.com/articles/10.1186/s40164-018-0124-7

  The epidemiology is well described in the western world but not in Asian countries like Malaysia. […] A total of 1010 patients were registered over a period of 5 years. […] The ethnic distribution revealed that Chinese had a relatively high weighted incidence proportion (43.2%), followed by Indian (23.8%), Malay (15.8%) and other ethnic groups (17.2%). […] Significant differences in platelet and WBC count were detected in ethnic groups and MPN sub-types. […] Among the risk factors, previous thrombosis, old age (60 years) and hypertension were significantly correlated to positive JAK2 V617F mutation. […] The presence of JAK2 V617F mutation is associated with higher risk of arterial thrombosis. […] Chinese ethnicity is associated with higher rates of MPN. […] The history of thrombosis, age 60 years and hypertension are risk factors that can be correlated to JAK2 V617F mutation.

• #16 Secondary Thrombocytosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560810/

  Secondary thrombocytosis is more common than primary thrombocytosis. Approximately 75% of individuals without prior myeloproliferative disorders develop thrombocytosis after splenectomy. In cases of iron deficiency anemia, the prevalence of reactive thrombocytosis is about 30%. Additionally, a Chinese study found that around 25.9% of children with respiratory tract infections had a platelet count of more than or equal to 50010/L. […] In an Italian study, 50% of children aged 1 to 24 months who were hospitalized for community-acquired infections developed thrombocytosis. Typically, preterm infants have lower platelet counts at birth, while term and late-preterm infants often experience thrombocytosis that peaks shortly after birth and then declines within the first month. High platelet counts in young children are commonly associated with respiratory infections and, less frequently, autoimmune diseases. A specific race, sex, or age has not been found to have an increased prevalence of secondary thrombocytosis.

• #17 Secondary Thrombocytosis: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/206811-overview

  Secondary thrombocytosis (reactive thrombocytosis) is a relatively common condition. The incidence varies with the underlying condition. The incidence of postsplenectomy secondary thrombocytosis is approximately 75-82%. […] In a retrospective review of patients with iron deficiency anemia, the prevalence of reactive thrombocytosis was found to be 31%. […] Overall, secondary thrombocytosis occurs in 3-13% of hospitalized children. However, in a Greek study of children 10 days to 8 years old who were hospitalized with viral pneumonia, and an Italian study of children 1 to 24 months old who were hospitalized for community-acquired infections, approximately half had thrombocytosis. […] Secondary thrombocytosis is more common than primary thrombocytosis. In a series from a large US university hospital that included 280 patients with extreme thrombocytosis (platelet count 1,000 109/L or greater), 82% had secondary thrombocytosis. […] No race predilection exists for secondary thrombocytosis. No sex predilection exists for secondary thrombocytosis, except that iron deficiency is more prevalent in females during childbearing years. No age predilection exists for secondary thrombocytosis.

• #18 Thrombocytosis and Thrombocytopenia Care In NJ, CT, and MD| Regional Cancer Care Associates

  https://www.regionalcancercare.org/hematology/benign-hematology-services/thrombocytosis-and-thrombocytopenia/

  Thrombocytosis is a condition that causes the body to produce an unusually high number of blood platelets. This may lead to more serious problems, such as a stroke or heart attack. The condition most commonly affects older individuals, and may or may not require treatment depending on its severity. There are two main types of thrombocytosis: […] Primary thrombocytosis: This type of thrombocytosis occurs for no apparent reason. The bone marrow creates abnormal and abundant blood cells, making it difficult for the body to form clots after a cut. This may be due to a gene mutation that was not inherited but developed after birth. […] Secondary thrombocytosis: This type of thrombocytosis develops in the setting of other medical issues, such as surgery, trauma, blood loss, inflammatory conditions, cancer, infections, or iron deficiency.

• #19 SciELO Brazil – Thrombocytosis in children Thrombocytosis in children

  https://www.scielo.br/j/ramb/a/6mrqnwkZgD8GpRJ6scL93wR/

  In our study, infections were the most common cause of thrombocytosis. […] The most common cause of secondary thrombocytosis was infection, accounting for 37.9% of all patients. […] Considerable differences were described in the epidemiology and the clinical presentation of thrombocytosis in children when compared to adults. […] The incidence of essential thrombocytosis is 0.62.5 per 100,000. […] The most common cause of thrombocytosis in our study was infections, while SCA and IDA were the other most common respective causes of secondary thrombocytosis.

• #20 Clinical relevance of thrombocytosis in primary care: a prospective cohort study of cancer incidence using English electronic medical records and cancer registry data | British Journal of General Practice

  https://bjgp.org/content/67/659/e405

  Thrombocytosis (raised platelet count) is an emerging risk marker of cancer, but the association has not been fully explored in a primary care context. […] To examine the incidence of cancer in a cohort of patients with thrombocytosis, to determine how clinically useful this risk marker could be in predicting an underlying malignancy. […] A total of 1098 out of 9435 males with thrombocytosis were diagnosed with cancer (11.6%; 95% confidence interval [CI] = 11.0 to 12.3), compared with 106 of 2599 males without thrombocytosis (4.1%; 95% CI = 3.4 to 4.9). A total of 1355 out of 21 826 females with thrombocytosis developed cancer (6.2%; 95% CI = 5.9 to 6.5), compared with 119 of 5370 females without (2.2%; 95% CI = 1.8 to 2.6). […] Thrombocytosis is a risk marker of cancer in adults; 11.6% and 6.2% cancer incidence in males and females, respectively, is worthy of further investigation for underlying malignancy.

• #21 Clinical relevance of thrombocytosis in primary care: a prospective cohort study of cancer incidence using English electronic medical records and cancer registry data | British Journal of General Practice

  https://bjgp.org/content/67/659/e405

  This study aimed to address that gap. […] The positive predictive value of thrombocytosis is 11.6% (95% confidence interval [CI] = 11.0 to 12.3) for males and 6.2% (95% CI = 5.9 to 6.5) for females. […] These figures are well above the 3% threshold used by the National Institute for Health and Care Excellence for suspected cancer investigation, and strongly suggest that cancer should be considered in patients showing thrombocytosis. […] This large-scale cohort study is the first from primary care to report the overall risk of cancer in patients with thrombocytosis, compared with those with normal platelet counts. […] The incidence of cancer rose with age and with a higher platelet count, and at least one-third of patients with lung and colorectal cancer with pre-diagnosis thrombocytosis had no other symptoms indicative of malignancy.

• #22 European Crohn´s and Colitis Organisation – ECCO – P320 Thrombocytosis in acute inflammatory bowel disease: a useful biomarker?

  https://www.ecco-ibd.eu/publications/congress-abstracts/item/p320-thrombocytosis-in-acute-inflammatory-bowel-disease-a-useful-biomarker.html

  Our study shows significant differences between clinical and biological markers in patients with acute IBD flares compared with those with infectious gastroenteritis. […] In particular, thrombocytosis occurs in IBD flares but not in infectious gastroenteritis. […] This biomarker can be used to differentiate between these diagnoses and guide management.

• #23 Development of thrombocytosis in HIV+ drug users: impact of antiretroviral therapy – University of Miami

  https://scholarship.miami.edu/esploro/outputs/991031578038402976

  With the exception of hemolytic anemia, the potential hematological toxicity of antiretrovirals (ARV) and combination treatments in HIV treated individuals has not been well established. […] We report, for the first time, hematological toxicity defined as thrombocytosis in 9% of the HIV+ patients receiving highly active antiretroviral treatment (HAART) being followed in a nutritional clinical trial. […] Together, these results support the proposal that HAART may increase the risk of hematological dysfunction and impact the risk of cardiovascular disease.

• #24 Thrombocytosis in 24 horses (1989-1994). | Research Bank

  https://madbarn.com/research/thrombocytosis-in-24-horses-1989-1994/?srsltid=AfmBOoqB8f2TzS6qUppUHQNrfGu0h1M6BpCFQ3krlNhBhvIr7c5zBRhp

  The records of horses presented to the Veterinary Teaching Hospital of North Carolina State University College of Veterinary Medicine between January 1, 1989 and April 30, 1994 were evaluated to determine risk factors associated with thrombocytosis. […] Infectious/ inflammatory disorders were observed more commonly in horses with high platelet counts than in horses with normal platelet counts. […] In the final multivariable model, the variables with the strongest association with thrombocytosis included leukocytosis, anemia, and hyperfibrinogenemia. […] The strong association of thrombocytosis with infectious/inflammatory disorders, however, should lead clinicians to suspect these types of conditions in horses with high platelet counts. […] The study concluded that thrombocytosis rarely results in clinical problems in horses and doesn’t generally need specific antiplatelet therapy. […] However, the strong relationship between thrombocytosis and infectious/inflammatory disorders may prompt clinicians to consider these types of conditions in cases of horses with high platelet counts.

• #25 One thousand patients with essential thrombocythemia: the Florence-CRIMM experience | Blood Cancer Journal

  https://www.nature.com/articles/s41408-023-00968-7

  At a median follow-up time of 8 years (range, 0.0342.9), major thrombosis after diagnosis was documented in 143 (14%) patients, including 91 (9%) arterial and 65 (6%) venous events. Overall, the incidence of arterial events (1% patient-year) was higher than that of venous events (0.7% patient-year). Incidence rates of major arterial/venous thrombosis for JAK2, type 1/1-like CALR, type 2/2-like CALR, MPL-mutated and TN cases were 10%/8%, 10%/5%, 8%/4%, 14%/9% and 3%/2%, respectively. […] To evaluate risk factors for arterial and venous thrombosis at diagnosis and during follow-up, we explored the impact of a number of variables. Concerning arterial thrombosis at or prior to diagnosis, univariate analysis reported as being statistically significant older age (p0.001), male sex (p=0.01), a JAK2V617F mutated status (p0.001) and presence of at least one cardiovascular risk factor (p0.001); multivariate analysis confirmed the independent prognostic impact of older age (p0.001), JAK2 mutation (p=0.004) and 1 cardiovascular risk factor (p0.001), but not male sex (p=0.08). Conversely, neither platelet (p=0.18) nor leukocyte counts (p=0.77) were significant. Concerning venous thrombosis at or prior to diagnosis, the presence of JAK2 mutation was the only significant risk factor identified (p=0.02). No risk factor was identified as regarded the risk of hemorrhages at or prior to diagnosis.

• #26 Essential Thrombocytosis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/30099

  Essential thrombocytosis is an indolent disease, and the median survival is 18 years. The reported life expectancy of patients with essential thrombocytosis was as high as 33 years in patients younger than 60 years. Compared to polycythemia vera, the life expectancy of patients with essential thrombocytosis is superior. Even though essential thrombocytosis is an indolent disease, they have a poorer life expectancy compared to the general population due to thrombotic events that could complicate the disease. […] The most common cause of mortality and morbidity in patients with essential thrombocytosis is thrombosis. Thrombosis occurs 20% of the time, followed by hemorrhage, which is reported to be 10%, and the estimates of risk for conversion are less than 1%.

• #26 Essential Thrombocytosis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/30099

  Essential thrombocytosis is the most common type of myeloproliferative neoplasm. Reports are that 1.0 to 2.5 individuals per 100000 have essential thrombocytosis yearly. The prevalence was reported to be 38 to 57 per 100000 between 2008 and 2010 and mostly occurred in females. The incidence of essential thrombocytosis increases with age, with most patients presenting between the ages of 50 and 60. […] The WHO diagnostic criteria for ETT are as follows. The diagnosis is made if all 4 major criteria are met or the first 3 major criteria and the minor ones are satisfied. […] It is helpful to recognize which genetic mutation a patient has, whether it is JAK2, CALR, or MPL, because each of these mutations determines the clinical features, complications, and survival of myeloproliferative neoplasm. Research shows that genetic testing will help to determine the phenotype and prognosis of essential thrombocytosis. For instance, a patient with CALR mutations of essential thrombocytosis has a better prognosis.

• #27 Essential thrombocytosis: diagnosis, differential diagnosis, complications and treatment considerations of relevance for a cardiologist | springermedizin.de

  https://www.springermedizin.de/essential-thrombocytosis-diagnosis-differential-diagnosis-compli/24010900

  Essential thrombocytosis (ET) is a rare haematological malignancy, with an incidence rate of 1.52.5/100,000 per year. […] The current review summarises symptoms, (differential) diagnosis, complications and treatment considerations of ET of relevance for a cardiologist. […] ET is a rare chronic myeloid malignancy, having an incidence rate of 1.52.5/100,000 per year. […] From a population point of view, ET is one of the rare (1%) pathologies associated with ACS. […] From a haematological point of view, the incidence of ACS in patients with ET ranges from 2% to 31% in various studies. […] The prevalence of ET can be at least 2.1% in certain groups. […] The incidence of thrombotic complications ranges from 9% to 84% at diagnosis and from 7% to 32% during long-term follow-up. […] For haemorrhage these rates range from 4% to 63% at diagnosis and from 8% to 14% during follow-up.

• #28 Essential thrombocytosis: diagnosis, differential diagnosis, complications and treatment considerations of relevance for a cardiologist | mijn-bsl

  https://mijn.bsl.nl/essential-thrombocytosis-diagnosis-differential-diagnosis-compli/24011868

  Essential thrombocytosis (ET) is a rare haematological malignancy, with an incidence rate of 1.52.5/100,000 per year. […] The current review summarises symptoms, (differential) diagnosis, complications and treatment considerations of ET of relevance for a cardiologist. […] ET is a rare chronic myeloid malignancy, having an incidence rate of 1.52.5/100,000 per year. […] From a population point of view, ET is one of the rare (1%) pathologies associated with ACS. […] From a haematological point of view, the incidence of ACS in patients with ET ranges from 2% to 31% in various studies. […] From a cardiologists point of view, we recently showed that the prevalence of ET can be at least 2.1% in certain groups. […] The incidence of thrombotic complications ranges from 9% to 84% at diagnosis and from 7% to 32% during long-term follow-up.

• #29 Essential thrombocytosis: diagnosis, differential diagnosis, complications and treatment considerations of relevance for a cardiologist | springermedizin.de

  https://www.springermedizin.de/essential-thrombocytosis-diagnosis-differential-diagnosis-compli/24010900

  The highest risk for recurrent events is observed within the first 2 years after the first thrombotic event and slowly declines thereafter. […] These numbers clearly indicate that thrombotic complications surpass haemorrhagic complications, that both types of events often coincide with the initial diagnosis of ET and that treatment of ET (after diagnosis) markedly reduces, but does not annihilate, the chance of a second event occurring. […] Overall transformation rates of the three MPNs into leukaemia are in the order of ET (2.6%), PV (3.9%) and PMF (9.3%) in 20 years. […] Although ET is a rare haematological malignancy, its prevalence is much higher in patients presenting with either thrombotic or haemorrhagic events, such as patients seen by a cardiologist, neurologist or vascular surgeon.

• #30 One thousand patients with essential thrombocythemia: the Florence-CRIMM experience | Blood Cancer Journal

  https://www.nature.com/articles/s41408-023-00968-7

  Univariate analysis for arterial thrombosis-free survival (A-TFS) identified older age (p0.001), male sex (p=0.03), presence of JAK2 mutation (p=0.04), cardiovascular risk factor (p=0.002) and previous arterial event (p=0.006) as risk factors for arterial thrombosis during follow-up. In contrast, antiplatelet treatment (p=0.02; HR 0.3) and cytoreduction (p=0.001; HR 0.4) had a protective effect on future arterial events. […] Univariate analysis for venous thrombosis-free survival (V-TFS) identified the presence of JAK2 mutation (p=0.01) and previous venous event (p=0.04) as predictors of a subsequent venous thrombosis; conversely, cytoreductive therapy displayed a protective role on venous thrombosis during follow-up (p=0.006), whereas aspirin had a borderline significance (p=0.09). Multivariate analysis for V-TFS identified JAK2 mutation (HR 1.9; p=0.03) as an independent predictor of cytoreductive therapy (HR 0.3; p=0.01) as protective for future venous events.

• #31

  https://link.springer.com/article/10.1007/s12471-023-01757-4

  Overall rates of complications in various regions/settings and using different definitions of events during long-term follow-up in patients with ET are relatively high. […] The incidence of thrombotic complications ranges from 9% to 84% at diagnosis and from 7% to 32% during long-term follow-up. […] For haemorrhage these rates range from 4% to 63% at diagnosis and from 8% to 14% during follow-up. […] These numbers clearly indicate that thrombotic complications surpass haemorrhagic complications, that both types of events often coincide with the initial diagnosis of ET and that treatment of ET (after diagnosis) markedly reduces, but does not annihilate, the chance of a second event occurring. […] Age (60 years), previous events, the presence of JAK2V617F, leukocytosis and long-term thrombocytosis have been identified as major risk factors for thromboembolic complications.

• #32

  https://link.springer.com/article/10.1007/s12471-023-01757-4

  Importantly, the presence of extreme thrombocytosis (platelets 1000109/l) was associated with a lower risk of thrombosis. […] With regard to the risk of recurrent CV events, patients with ET have been stratified into very low-, low-, intermediate- and high-risk individuals. […] ET, similar to the other MPNs, can undergo several transformations. […] Finally, patients with MPNs have a higher risk of various second malignancies, e.g. of the skin, brain, kidney and endocrine organs. […] Although ET is a rare haematological malignancy, its prevalence is much higher in patients presenting with either thrombotic or haemorrhagic events, such as patients seen by a cardiologist, neurologist or vascular surgeon. […] Any specialist should be aware of such underlying pathology and thus actively screen for elevated thrombocytes (i.e. a platelet count 450109/l) in patients presenting with either thrombotic or haemorrhagic events, notably in those without concomitant CV risk factors.

• #33 Essential thrombocytosis: diagnosis, differential diagnosis, complications and treatment considerations of relevance for a cardiologist | mijn-bsl

  https://mijn.bsl.nl/essential-thrombocytosis-diagnosis-differential-diagnosis-compli/24011868

  For haemorrhage these rates range from 4% to 63% at diagnosis and from 8% to 14% during follow-up. […] Numerically, myocardial infarction is a rare complication of ET with an incidence rate ranging from 2% to 31% within various studies/regions/settings/periods/definitions. […] Overall transformation rates of the three MPNs into leukaemia are in the order of ET (2.6%), PV (3.9%) and PMF (9.3%) in 20 years. […] Although ET is a rare haematological malignancy, its prevalence is much higher in patients presenting with either thrombotic or haemorrhagic events, such as patients seen by a cardiologist, neurologist or vascular surgeon.

• #34

  https://link.springer.com/article/10.1007/s00277-021-04485-0

  However, whereas guidelines for a risk-adapted therapy are available for adults affected by ET, there are no consensus recommendations tailored to the pediatric population. […] Because of the differences in the epidemiology and clinical presentation of thrombocytosis in childhood when compared to adults, an algorithm specifically adapted to the pediatric population is clearly required for clinical practice. […] The occurrence of thromboembolic complications, one of the major concerns in persistent thrombocytosis, is far more common in primary than in secondary thrombocytosis. […] In young patients, malignant transformation is rarely observed. […] A recent literature review that analyzed 396 patients with ET and 75 patients with PV that were 20 years of age, respectively, found fibrotic transformation in 2% of patients and no development of acute leukemia.

• #35

  https://haematologica.org/article/view/haematol.2024.285329

  Familial thrombocythemia refers to an ultra-rare inherited syndrome that is characterized by elevated platelet counts (450×109/L). […] Our case report now identifies JAK2F556V as a germline mutation in a family with elevated platelet counts. This adds a significant new aspect to the existing literature by elucidating the hereditary nature of JAK2 mutations within familial thrombocythemia. […] In summary, the present study shows that the JAK2 F556V mutation can occur as a germline variant and can be passed on to the offspring. In the present family, this heterozygous germline mutation led to thrombocytosis. Surveillance of family members because of a potential increased risk for any other blood cancer might be discussed.

• #36

  https://link.springer.com/article/10.1007/s12471-023-01757-4

  Essential thrombocytosis (ET) is a rare haematological malignancy, with an incidence rate of 1.52.5/100,000 per year. […] The current review summarises symptoms, (differential) diagnosis, complications and treatment considerations of ET of relevance for a cardiologist. […] ET is a rare chronic myeloid malignancy, having an incidence rate of 1.52.5/100,000 per year. […] From a population point of view, ET is one of the rare (1%) pathologies associated with ACS. […] From a haematological point of view, the incidence of ACS in patients with ET ranges from 2% to 31% in various studies. […] The prevalence of ET can be at least 2.1% in certain groups. […] Increased routine laboratory screening within the general population will increase the incidental finding of thrombocytosis. […] At present, thrombocytosis is encountered in 1.52.2% of people consulting primary care.

• #37 Thrombocytosis and Thrombocytopenia Care In NJ, CT, and MD| Regional Cancer Care Associates

  https://www.regionalcancercare.org/hematology/benign-hematology-services/thrombocytosis-and-thrombocytopenia/

  As noted, symptoms of thrombocytosis and thrombocytopenia can be mild or non-existent. This is why many people receive a diagnosis after having a complete blood count (CBC) test for other reasons. The test shows abnormal platelet levels in the blood, alerting doctors of a potential blood disorder. […] For those with thrombocytosis, physicians may conduct further genetic testing or look for signs of undiagnosed cancer.

• #38 Essential thrombocythemia | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/essential-thrombocythaemia?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjgh&lang=us

  Essential thrombocythemia has an incidence of ~1 (range 1.2-3.0) per 100,000. It is most common in the 55-65 years age group with a female predominance. […] The disease has traditionally been a diagnosis of exclusion, requiring the absence of reactive conditions and other clonal disorders that may present with thrombocytosis.

• #39 Epidemiology of immune thrombocytopenia: study of adult patients at a referral hematology service in Northeastern Brazil | Hematology, Transfusion and Cell Therapy

  https://www.elsevier.es/pt-revista-hematology-transfusion-cell-therapy-396-avance-resumen-epidemiology-immune-thrombocytopenia-study-adult-S2531137923025853?referer=buscador

  The results of this survey on the epidemiology of ITP in Brazil suggest that ethnic and geographical factors may have a great impact on age and sex distribution and on the distribution of secondary ITP. […] Although ITP has been studied for over a century, its epidemiology is not well known for countries like Brazil. In fact, no data on the epidemiology of ITP in Brazil is available as according to the March 2019 report issued by the National Commission for the Incorporation of Technology into the Brazilian National Health Service (CONITEC), the incidence and prevalence of ITP in the Brazilian population have not yet been estimated. […] This study of the epidemiology of ITP in Brazil is intended as a contribution to public health policy development, health care cost modeling and the updating of curricula used in health education. Based on a sample of 187 adult patients, the demographic profile drawn in this study shares similarities with profiles from other countries, especially France, with regard to the prevalence of the female sex and age distribution. These similarities between the profiles of Cear and France may be related to genetic inheritance. Secondary ITP displayed a bimodal distribution and a linear increase between 38 and 68 years of age.

• #40

  https://haematologica.org/article/view/haematol.2022.281388

  Risk stratification for thrombosis in ET patients has been mostly based on the presence of conventional risk factors, such as age 60 years and a positive history of thrombosis. The revised International Prognostic Score for Thrombosis in ET (IPSET-thrombosis) model also considered the presence of a JAK2 mutation and of additional cardiovascular risk factors to stratify ET patients into four categories of thrombosis risk. […] Prevention of recurrent thrombosis in ET patients must follow the general guidelines for secondary prevention in the general population, including antiplatelet agents and anticoagulant drugs, depending on the clinical setting. Primary prevention of thrombosis is based on the prophylactic use of low-dose aspirin (81-100 mg daily) and cytoreduction. Aspirin is recommended for all risk categories, with the exception of patients at very low risk, who should only be given aspirin for treatment of vasomotor/microvascular disturbances.

• #41 Essential Thrombocythemia Market to Witness Upsurge in

  https://www.globenewswire.com/news-release/2025/03/05/3037669/0/en/Essential-Thrombocythemia-Market-to-Witness-Upsurge-in-Growth-During-the-Study-Period-2020-2034-DelveInsight.html

  Additionally, the market is dominated by the off-label use of existing treatments, such as hydroxyurea and interferon, reducing the urgency for novel therapies. […] Moreover, essential thrombocythemia treatment poses a significant economic burden and disrupts patients overall well-being and QOL. […] Furthermore, the essential thrombocythemia market growth may be offset by failures and discontinuation of emerging therapies, unaffordable pricing, market access and reimbursement issues, and a shortage of healthcare specialists. […] In addition, the undiagnosed, unreported cases and the unawareness about the disease may also impact the essential thrombocythemia market growth.

• #42 Essential Thrombocythemia Market to Witness Upsurge in

  https://www.globenewswire.com/news-release/2025/03/05/3037669/0/en/Essential-Thrombocythemia-Market-to-Witness-Upsurge-in-Growth-During-the-Study-Period-2020-2034-DelveInsight.html

  The essential thrombocythemia epidemiology section provides insights into the historical and current essential thrombocythemia patient pool and forecasted trends for the 7MM. […] The essential thrombocythemia market report proffers epidemiological analysis for the study period 20202034 in the 7MM segmented into: Total Diagnosed Prevalent Cases, Symptom-specific Cases, Gender-specific Cases, Mutation-specific Cases, Risk-specific Cases, Age-specific Cases, Total Treated Cases. […] The anticipated introduction of emerging therapies with improved efficacy and a further improvement in the diagnosis rate is expected to drive the growth of the essential thrombocythemia market in the 7MM. […] However, several factors may impede the growth of the essential thrombocythemia market. One major challenge is the limited patient population, as essential thrombocythemia is a rare myeloproliferative neoplasm, making it less attractive for large pharmaceutical investments.

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