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Essential thrombocythaemia is a slow blood cancer in which the marrow makes too many platelets. Most people need only aspirin and monitoring; those at higher risk of clots take a drug to lower the platelet count, usually hydroxyurea or interferon, with anagrelide in reserve.
Essential thrombocythaemia is a classical myeloproliferative neoplasm defined by a sustained platelet count above 450 x 10^9/L with a marrow full of large, mature megakaryocytes and no other explanation. About 60 percent carry JAK2 V617F, 20 to 25 percent a CALR mutation and 3 to 5 percent an MPL mutation; the rest are triple negative. Many people have no symptoms and are found on a routine blood count; others have headaches, visual disturbance, burning red hands and feet (erythromelalgia) or, at high platelet counts, paradoxical bleeding. Treatment is set by the IPSET-thrombosis score, which weighs age, prior clot, JAK2 status and cardiovascular risk: very low-risk patients may need nothing, low-risk patients take low-dose aspirin, and high-risk patients add cytoreduction with hydroxyurea or interferon, with anagrelide second line. The PT-1 trial showed hydroxyurea plus aspirin beats anagrelide plus aspirin on arterial clots and bleeding. Progression to myelofibrosis happens in a minority over decades and to acute leukaemia in a few percent. Bomedemstat, an LSD1 inhibitor, is in a phase 3 trial against hydroxyurea, and antibodies against mutant CALR are the first treatments aimed at the clone itself.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
Full blood count, JAK2, CALR and MPL testing, bone marrow biopsy to confirm ET and exclude prefibrotic myelofibrosis, and exclusion of reactive causes (iron deficiency, inflammation, infection, splenectomy).
Observation alone in very low risk (under 60, no clot, JAK2-negative); low-dose aspirin for low risk and for anyone with microvascular symptoms, once acquired von Willebrand deficiency is excluded at very high platelet counts.
Cytoreduction to a platelet count under 400 x 10^9/L: hydroxyurea first line (PT-1), pegylated or ropeginterferon alfa-2b preferred under 60 and in pregnancy, anagrelide second line.
Switch to interferon or anagrelide; ruxolitinib did not beat best available therapy in MAJIC-ET but relieves symptoms.
Managed as myelofibrosis: JAK inhibitors for spleen and symptoms, transplant for fit higher-risk patients.
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Query for this cancer: (TITLE:"Essential thrombocythaemia" OR ABSTRACT:"Essential thrombocythaemia" OR TITLE:"Essential thrombocythemia" OR ABSTRACT:"Essential thrombocythemia" OR TITLE:"Primary thrombocythaemia" OR ABSTRACT:"Primary thrombocythaemia" OR TITLE:"Essential thrombocytosis" OR ABSTRACT:"Essential thrombocytosis") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Essential thrombocythaemia (ET), not a curated reading list.
The first account of a primary platelet disorder with bleeding and clotting.
In 809 high-risk patients hydroxyurea plus aspirin gave fewer arterial clots, less bleeding and less progression to myelofibrosis than anagrelide plus aspirin.
Klampfl and Nangalia find CALR exon 9 mutations in most JAK2-negative ET and myelofibrosis.
Marrow histology now distinguishes true ET from early myelofibrosis, which carries a worse outlook.
Ruxolitinib matched but did not beat best available therapy after hydroxyurea failure, though it eased symptoms.
Merck starts the Shorespan-007 trial against hydroxyurea in high-risk ET.
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Reduce dose for CrCl below 60 with platelets under 150; dialysis dosing after each session.
Intense itching, prickling or burning of the skin within minutes of contact with water, typically after a shower. It affects a large minority of people with polycythaemia vera and can be the most disabling symptom.
Burning pain, redness and heat in the hands or feet, brought on by warmth. In polycythaemia vera and essential thrombocythaemia it comes from platelets clumping in tiny vessels and often disappears with low-dose aspirin.
See all on the product pages:AspirinRopeginterferon alfa-2bRuxolitinib·Printable cards in the navigator
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