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Thrombocythemia 2

ET


Presentation

  • On admission, the patient presented a haemoglobin count of 11.6 g/dL and 569 000 platelets.[revespcardiol.org]
  • In a previous report by the present authors, the presence of the JAK2 V617F mutation in CML was reported to be rare, with the present case being the only one detected among 55 cases of CML analyzed ( 17 ).[spandidos-publications.com]
  • Acquired V617F JAK2 mutation present B1. No cause for a reactive thrombocytosis normal inflammatory indices B2. No evidence of iron deficiency stainable iron in the bone marrow or normal red cell mean corpuscular volume B3.[en.wikipedia.org]
  • Acta Haematol. 1991 ; 85 : 31 – 33. 2 Simultaneous presentation of chronic lymphocytic leukosis and essential thrombocythemia (in Spanish). An Med Interna. 1995 ; 12 : 566. 3.[academic.oup.com]
  • Revised criteria: JAK2 clonal marker present (or if not present, the above reactive causes excluded). JAK 2 is present in about 50% of cases of ET.[clinicaladvisor.com]
Pathologist
  • […] neoplasm ET essential thrombocythemia B-CLL B-cell chronic lymphocytic leukemia JAK2 Janus Kinase 2 STAT signal transducers and activators of transcription FISH fluorescent in situ hybridization IL-1α interleukin-1 alpha American Society of Clinical Pathologists[academic.oup.com]
Spontaneous Hemorrhage
  • Clinical syndrome characterized by repeated spontaneous hemorrhages and a remarkable increase in the number of circulating platelets.[icd10data.com]

Workup

  • Polycythemia Vera Workup. Medscape Drugs and Diseases. Available online at . Accessed on 1/22/17. Lal, A. (2016 November 10 Updated). Essential Thrombocytosis Workup. Medscape Drugs and Diseases. Available online at .[labtestsonline.it]
  • The blood count was part of the preoperative workup for circumcision and right side orchidopexy. The protocol for childhood thrombocytosis, shown in Fig. 1, is started.[elsevier.es]
  • Of patients with Budd-Chiari syndrome (hepatic-inferior vena cava obstruction), 10% to 13% have coexisting PV; therefore, testing for the presence of JAK2 V617F mutation is part of the routine workup for unexplained liver thrombosis.[clevelandclinicmeded.com]

Treatment

  • Finally, we discuss the decision to initiate treatment and assess the quality of evidence supporting the use of established, available therapies as well as novel treatments.[scholars.northwestern.edu]
  • What Are the Available Treatments for ET? As with other MPNs, there is no single treatment option that is appropriate or effective for all ET sufferers.[mpnresearchfoundation.org]
  • During long-term treatment, subjective side effects were tolerable, especially using 3 mu three times a week. We conclude that IFN alpha-2b is an effective drug in the long-term treatment of ET.[link.springer.com]
  • After discontinuation of treatment, increases in platelet counts were consistent with the maintenance interval during treatment.[nejm.org]
  • Ruxolitinib versus Standard Therapy for the Treatment of Polycythemia Vera. N Engl J Med. 2015;372:426-435.[targetedonc.com]

Prognosis

  • The data collected indicate the importance of CALR mutation in prognosis of ET patients.[cellular-molecular-medicine.imedpub.com]
  • Let’s Change Your ET Prognosis Together. A blood cancer diagnosis can be emotionally challenging. But the good news is that you don’t have to fight ET alone. At the MPN Research Foundation, we’re here to help change your ET prognosis.[mpnresearchfoundation.org]
  • Compared with JAK2 mutant ET, CALR mutant ET showed a different clinical manifestation and an unfavorable prognosis. Thus, CALR is a potentially valuable diagnostic marker and therapeutic target in ET.[ncbi.nlm.nih.gov]
  • Introduction Neoplasia of mature myeloid cells (mostly megakaryocytes ) typically high levels of erythrocytes, granulocytes are also seen Caused by a JAK2 kinase mutation Seen mainly in older adults (5th-6th decade) Presentation Symptoms bleeding Prognosis[medbullets.com]
  • Prognosis The large majority of the patients survive 10 years. No significant difference between life expectancy of ET patients and age-matched subjects was observed in a study.[atlasgeneticsoncology.org]

Etiology

  • They are organised into groups, and further divided into clinical, etiological or histopathological sub-types.[orpha.net]
  • There are very few cases of co-existent CLL and ET reported in the literature. 1–5 The etiology of this co-occurrence could be multifold.[academic.oup.com]
  • For the condition to be labeled a reactive thrombocytosis, a credible underlying etiology must be identified.[asams.org]
  • Figure 3 Etiology and pathophpysiology of platelet-mediated fibromuscular intimal proliferation and platelet thrombi in erythromelalgia and its acrocyanotic complications in thrombocythemia vera (essential thrombocythemia and polycythemia vera) and etiology[wjgnet.com]
  • Etiology of thrombocytosis in a general medicine population: analysis of 801 cases with emphasis on infectious causes. J Clin Med Res. 2012; 4(6):415-423. 24. Kondo T, Okabe M, Sanada M, et al.[intramed.net]

Epidemiology

  • […] thrombocythemia (effective October 01, 2015) Signs and Symptoms Bleeding from mucosa l surfaces Burning or tingling in the hands or feet GI bleeding Headache Redness and warmth of the hands or feet Splenomegaly Thrombosis Progression and Transformation None Epidemiology[seer.cancer.gov]
  • Although many factors play a key role in the risk of developing MPDs, according to published epidemiological studies the most important risk factors include: age, sex, cancer treatment and exposure to petrochemicals.[oatext.com]
  • Epidemiology ET has an annual incidence of 1.5 to 2.4 patients /100,000. The disease incidence may show a peak around 30 years in females, with a second peak in the elderly with a 1:1 male-to-female ratio.[atlasgeneticsoncology.org]
  • Epidemiological, diagnostic, therapeutic and prognostic aspects of essential thrombocythemia in a retrospective study of the GIMMC group in two thousand patients [abstract]. Blood. 1997. 90(suppl 1):348a.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • The description of this mutation raised the question of its true role in MPD pathophysiology.[hematologyandoncology.net]
  • “Myeloproliferative neoplasms: molecular pathophysiology, essential clinical understanding, and treatment strategies”. J Clin Oncol.. vol. 29. 2011. pp. 573-582. [Comprehensive review of pathophysiology with clinical correlations.][cancertherapyadvisor.com]
  • Genetic basis and molecular pathophysiology of classical myeloproliferative neoplasms. Blood. 2017;129:667-679. Rumi E, Cazzola M. Diagnosis, risk stratification, and response evaluation in classical myeloproliferative neoplasms.[labtestsonline.it]
  • The past few decades have witnessed significant advances in the understanding of the etiology, diagnosis and treatment of essential thrombocythemia (ET), but more detailed insights are required to understand the pathophysiologic mechanism of the disease[oatext.com]
  • […] thrombocytosis is characterized by the following [4, 5, 6, 7, 8, 9, 10] : A persistently elevated platelet count greater than 450,000/µL Megakaryocytic hyperplasia Splenomegaly A clinical course complicated by thrombotic or hemorrhagic episodes or both Pathophysiology[emedicine.medscape.com]

Prevention

  • Introduction Neoplasia of mature myeloid cells (mostly megakaryocytes ) typically high levels of erythrocytes, granulocytes are also seen Caused by a JAK2 kinase mutation Seen mainly in older adults (5th-6th decade) Presentation Symptoms bleeding Prognosis, Prevention[medbullets.com]
  • Although ANA, when compared to HC, appears to be slightly less effective in preventing arterial thrombosis and myelofibrotic transformation, it is associated with lower risk of venous thrombosis.[dovepress.com]
  • It can rarely transform to acute myeloid leukemia, and there is no treatment to prevent the transformation. Since you have no symptoms, the main risks to you are abnormal blood clotting and bleeding.[detroitnews.com]
  • The role of Asp in the prevention of arterial thromboses has been clearly established in the general population.[ojrd.biomedcentral.com]

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