primary myelofibrosis

primary myelofibrosis

chronic, eventually fatal myeloproliferative neoplasm in which normal bone marrow hematopoietic tissue is replaced by reticulin fibers and blood cell production moves to other organs, called myeloid metaplasia. The peripheral blood film presents all stages of myelocytic and erythrocytic maturation and teardrop-shaped red blood cells called dacryocytes.

chronic idiopathic myelofibrosis

A chronic progressive condition characterised by panmyelosis and variable marrow fibrosis, massive splenomegaly secondary to extramedullary haematopoiesis, and leukoerythroblastic anaemia with dysmorphic red blood cells, circulating normoblasts, immature white blood cells and atypical platelets.
 
Clinical findings
Patients are often > age 50, suffer from insidious weight loss, anaemia, and abdominal discomfort due to splenomegaly, often with hepatomegaly; 80% have nonspecific chromosome defects.
 
Diagnosis
Bone marrow biopsy.
 
Management
No specific therapy; packed RBCs for anaemia; androgens may reduce transfusion requirements, but are poorly tolerated in women; recombinant erythropoietin.
 
Prognosis
Survival ± 5 years, often progresses to acute leukaemia.

Terminology
No name used for this condition has proven consistently satisfactory to those who work in the field. Chronic idiopathic myelofibrosis is preferred by the World Health Organisation, while others prefer the term primary myelofibrosis. None of the terms fully take into account the functional defects—e.g., haemopoietic stem cell disturbance, extramedullary haemopoiesis and the pathological changes seen in the bone marrow (e.g., intense marrow fibrosis).
References in periodicals archive ?
Primary myelofibrosis is classified in oncology under myoproliferative neoplasms.
Correction of anemia in a transfusion-dependent patient with primary myelofibrosis receiving iron chelation therapy with deferasirox (Exjade, ICL670).
CTI BioPharma Corp (NASDAQ and MTA: CTIC), a biopharmaceutical company, has announced today that it has enrolled its first patient in PAC203, a Phase two clinical trial of pacritinib in patients with primary myelofibrosis who have failed prior ruxolitinib therapy.
The three main types of MPNs are primary myelofibrosis (PMF), polycethemia vera (PV) and essential thrombocythemia (ET).
A diagnosis of paediatric primary myelofibrosis was made on biopsy.
Primary myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by abnormal proliferation of megakaryocytes, bone marrow fibrosis, and extramedullary hematopoiesis.
1) Between 90% and 98% of patients with polycythemia vera and about 50% of patients with essential thrombocythemia (ET) and primary myelofibrosis (PMF) harbor the JAK2V617F mutation.
Moreover, we have been able to show that the approach empowers the therapeutic decision-making capability of hema-oncologists in a concrete clinical setting using primary myelofibrosis and lymphoma as test diseases.
A diagnosis requires meeting all the following criteria:[sup][1] platelet (PLT) count ≥450 x 10[sup]9/L; megakaryocyte proliferation with large and mature morphology; not meeting the WHO criteria for chronic myeloid leukemia, polycythemia vera, primary myelofibrosis, myelodysplastic syndromes, or other myeloid neoplasm; demonstration of JAK2V617F or other clonal markers or no evidence of reactive thrombocytosis.
Mutations in the CALR (calreticulin) gene were recently identified in most patients with essential thrombocythemia or primary myelofibrosis who lack JAK2 mutations.
Ruxolitinib was initially approved in 2011 for the treatment of intermediate- and high-risk myelofibrosis, including primary myelofibrosis, post-polycythemia vera myelofibrosis, and post-essential thrombocythemia myelofibrosis.
INTRODUCTION: It is one among the 4 Myeloproliferative disorders others are Polycythemia vera, Chronic Myeloid leukemia, Primary myelofibrosis.

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