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Primary myelofibrosis is a blood cancer in which the marrow scars over, the spleen swells and patients become anaemic and exhausted. JAK inhibitors, ruxolitinib first (COMFORT) and then fedratinib, pacritinib and momelotinib (MOMENTUM), shrink the spleen and relieve symptoms; only a donor stem cell transplant can cure it.
Primary myelofibrosis arises from a haematopoietic stem cell carrying JAK2 V617F (about 60 percent), CALR (about 25 percent) or MPL (5 to 8 percent), or none of the three (triple negative, the worst group), with additional high-risk mutations in ASXL1, SRSF2, EZH2, IDH1/2 and U2AF1. The clone drives cytokine release and megakaryocyte abnormalities that scar the marrow; blood production moves to the spleen and liver, which enlarge, and patients develop anaemia, constitutional symptoms, early satiety and bone pain. A prefibrotic phase resembles essential thrombocythaemia. Risk scores (IPSS, DIPSS-plus, MIPSS70 and MIPSS70-plus v2 with mutations and cytogenetics) predict survival and steer the transplant decision; about one in five progress to blast phase, for which no treatment reliably works.
JAK inhibitors treat the disease's consequences. Ruxolitinib, the first, shrank the spleen by 35 percent or more in 41.9 percent of patients against 0.7 percent on placebo at 24 weeks in COMFORT-I (2012), and beat best available therapy in COMFORT-II, with symptom scores halving in nearly half; approval came in 2011 and a survival advantage emerged in pooled long-term data. Fedratinib (JAKARTA, 2019 approval) works after ruxolitinib failure but carries a warning for Wernicke encephalopathy; pacritinib (PERSIST-2, approved 2022) is the option when platelets fall below 50 x 10^9/L; and momelotinib, which also blocks ACVR1 and so raises haemoglobin, was approved in 2023 after MOMENTUM, in which 25 percent of anaemic, previously treated patients had their symptom score halve against 9 percent on danazol, with more becoming transfusion independent. None of them clears the clone.
Allogeneic transplant is the only cure and is offered to fit patients with higher-risk disease (MIPSS70 high, typically under 70), with a JAK inhibitor to shrink the spleen beforehand; transplant-related mortality is substantial, and timing is the central clinical judgement. Anaemia is managed with momelotinib, erythropoietin, danazol, transfusion and, in trials, luspatercept (INDEPENDENCE). Combinations of ruxolitinib with navitoclax (TRANSFORM-1) or the BET inhibitor pelabresib (MANIFEST-2) roughly doubled spleen responses in phase 3 but have not yet reached approval, imetelstat is being tested against survival itself in IMpactMF, and interferon, selinexor and anti-fibrotic approaches are in trials; whether any drug changes the disease's course rather than its symptoms is the field's central question.
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), Essential thrombocythaemia (ET), 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, 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
Observation; aspirin for thrombosis risk where platelets are high; hydroxyurea or interferon for proliferative features.
Ruxolitinib (COMFORT-I and II) first; fedratinib after ruxolitinib failure or intolerance; pacritinib if platelets are below 50 x 10^9/L; momelotinib if anaemic.
Momelotinib (MOMENTUM), erythropoiesis-stimulating agents where erythropoietin is low, danazol, transfusion with iron chelation; luspatercept in trials (INDEPENDENCE).
Allogeneic stem cell transplant, usually under 70 and at MIPSS70 high or DIPSS intermediate-2 or higher, after JAK inhibitor to reduce spleen size.
Switch JAK inhibitor; combination trials of ruxolitinib with navitoclax, pelabresib, selinexor or imetelstat; imetelstat versus best available therapy in IMpactMF.
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MOMENTUM addressed the biggest gap left by ruxolitinib: patients whose anaemia makes standard JAK inhibition hard to give. Momelotinib is now the preferred option for anaemic, previously treated myelofibrosis and is being adopted in first line for anaemic patients. The absolute symptom benefit is modest and durable disease modification has not been shown.
With COMFORT-I, the evidence for ruxolitinib as the first approved treatment for myelofibrosis and for JAK inhibition as the standard for spleen and symptom control.
COMFORT-I turned the 2005 discovery of the JAK2 V617F mutation into the first effective medicine for myelofibrosis, transforming symptom control for a disease with no prior standard. Ruxolitinib is still the reference first-line therapy, with fedratinib, pacritinib and momelotinib as alternatives for cytopenic patients. It does not eliminate the malignant clone or reverse fibrosis in most patients.
Query for this cancer: (TITLE:"Primary myelofibrosis" OR ABSTRACT:"Primary myelofibrosis" OR TITLE:"PMF" OR ABSTRACT:"PMF" OR TITLE:"Myelofibrosis" OR ABSTRACT:"Myelofibrosis" OR TITLE:"Chronic idiopathic myelofibrosis" OR ABSTRACT:"Chronic idiopathic myelofibrosis" OR TITLE:"Agnogenic myeloid metaplasia" OR ABSTRACT:"Agnogenic myeloid metaplasia" OR TITLE:"Post-PV and post-ET myelofibrosis secondary myelofibrosis" OR ABSTRACT:"Post-PV and post-ET myelofibrosis secondary myelofibrosis") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Primary myelofibrosis, not a curated reading list.
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Nausea and anorexia are near-universal: prophylactic 5-HT3 antagonist plus olanzapine or dexamethasone.
Reduce dose for CrCl below 60 with platelets under 150; dialysis dosing after each session.
A shortage of red blood cells or haemoglobin, causing tiredness and breathlessness. In cancer it comes from the disease itself (marrow infiltration, bleeding, inflammation), from chemotherapy suppressing the marrow, and from some targeted drugs.
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:AspirinImetelstatLuspaterceptRopeginterferon alfa-2bRuxolitinibSelinexor·Printable cards in the navigator
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