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MYELOFIBROSIS
  • It is myeloproliferative disorder
  • Other myeloproliferative conditions are
    • Essential thrombocytosis
    • Polycythemia Rubra vera
    • Chronic myelogenous leukemia
  • Although the condition is thought to be immunologically mediated, the cause of this condition is unknown
  • There is excessive proliferation of white cell precursors and platelet precursors called megakaryocytes
  • There is fibroblastic proliferation in the bone marrow. Areas of blood formation occurs in unusual parts of the body
  • Other findings include dysplastic megakaryocytes and increased collagen Types I and III in the marrow
  • Median age of diagnosis is 60 years but the disease can occur in any age group (one month of life to the ninth decade).
  • The disease may run in families
  • Median survival is about five years (range 1-15 years)

SIGNS AND SYMPTOMS

  • Some patients may be asymptomatic and diagnosis following a routine blood test is not unusual
  • Generalized weakness, secondary to anemia
  • Anemia
  • Rapid heart beat
  • Shortness of breath
  • Weight loss
  • Enlarged spleen and liver
  • Bleeding
  • Bone pains, osteosclerosis
  • Neutrophilic dermatosis - skin infiltration by neutrophils
  • Pyoderma gangrenosum; infections
  • Stroke, paralysis coma and other neurological conditions may occur
  • It is often difficult to aspirate marrow
  • Silver stain of the marrow shows increased reticulin fibers
  • In the "spent" phase of the disease, the platelets, white and red cells may be decreased but there is still evidence of increased activity of platelet precursor - megakaryocytes
  • Myelofibrosis may occur as a terminal phase of Polycythemia

TREATMENT

  • Treatment may not be needed in stable disease
  • Interferon alpha and interferon gamma act synergistically
  • Androgens and Glucocorticoids may also be used
  • Chemotherapy - Hydroxyurea and Busulfan may be used to reduce counts
  • Radiation therapy may be indicated in the following situations
    • Splenic pain following infarction of the spleen
    • Massive splenic enlargement
    • Ascites secondary to myeloid metaplasia of the peritoneum
    • Focal areas of bone pain
    • Areas of Myelofibrosis outside the marrow, especially if it occurs in the spine giving neurological deficits
  • Splenectomy (removal of the spleen) is necessary in situations where the patient receives multiple transfusions, painful enlarged spleen, thrombocytopenia and Portal hypertension
  • Vitamin D
  • Marrow curettage
  • Inhibitors of collagen synthesis e.g., monoamine, oxidase inhibitors and dehydroproline (chelator)
  • Bone marrow transplantation may be effective in patients under 40 years.