chronic idiopathic myelofibrosis

(redirected from osteomyelosclerosis)

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).

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References in periodicals archive ?

It has been shown that adult NFATc2 knockout mice exhibit extramedullary hematopoiesis and suffer from osteomyelosclerosis and hypoplasia in the bone marrow (with significant losses of erythrocytes, lymphocytes, and megakaryocytes) [30].

Kujawski et al., "Osteomyelosclerosis, anemia and extramedullary hematopoiesis in mice lacking the transcription factor NFATc2," Haematologica, vol.

NFAT signaling in osteoblasts regulates the hematopoietic niche in the bone microenvironment

The reasons for failure of the technique with disposable spinal needle are the same as those found with conventional Islam Needle and are related to the type of blood disease (myelofibrosis, severe aplastic anaemia and osteomyelosclerosis).