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 (PMF) is a blood disorder in which the patient's normal bone marrow becomes acellular and fibrotic.
In addition, when there is leukoerythroblastosis in the peripheral blood along with increased fibrosis and increased atypical megakaryocytes and megakaryoblasts, then the differential diagnosis includes acute megakaryoblastic leukemia, acute panmyelosis with myelofibrosis, and transformed primary myelofibrosis.
The classic MPNs are polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF), and chronic myelogenous leukemia (CML).
JAK2 V617F mutational status predicts progression to large splenomegaly and leukemic transformation in primary myelofibrosis.
The WHO diagnostic criteria for polycythemia vera, essential thrombocythemia and primary myelofibrosis.
Nonetheless, they said that the association, coupled with the lack of efficacy and an enlargement of the spleen seen in some patients who were given ESA therapy, was enough to halt use of ESAs for primary myelofibrosis.
The most common BCRJABL1-negative MPNs are polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF).
An individual patient supply program for ruxolitinib for the treatment of patients with primary myelofibrosis, post-polycythemia vera myelofibrosis, or post-essential thrombocythemia myelofibrosis.
The discovery of the Philadelphia chromosome and its subsequent association with the BCR/ABL1 mutation led the way to the grouping of ET with the other BCR/ABL-negative myeloproliferative neoplasms, polycythemia vera (PV) and primary myelofibrosis (PMF).
As we have mentioned in the case report, most patients with primary myelofibrosis have karyotypic abnormalities at diagnosis and some of them have been associated with an adverse prognosis.
In the bone marrow of patients with primary myelofibrosis, there is proliferation and atypia of:
An open-label, multicenter, expanded access study assessing the safety and efficacy of oral ruxolitinib administered to patients with primary myelofibrosis, post-polycythemia myelofibrosis or post-essential thrombocythemia myelofibrosis.

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