agnogenic myeloid metaplasia

agnogenic myeloid metaplasia.

See myeloid metaplasia.

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metaplasia [met″ah-pla´zhah]

the change in the type of adult cells in a tissue to a form abnormal for that tissue. adj., adj metaplas´tic.

agnogenic myeloid metaplasia the primary or idiopathic form of myeloid metaplasia, which is often accompanied by myelofibrosis; it is considered one of the myeloproliferative disorders. Called also aleukemic or nonleukemic myelosis.

myeloid metaplasia the occurrence of myeloid tissue in extramedullary sites; specifically, a syndrome characterized by splenomegaly, anemia, nucleated erythrocytes and immature granulocytes in the circulating blood, and extramedullary hematopoiesis in the liver and spleen. The primary form is called agnogenic myeloid metaplasia. The secondary or symptomatic form may be associated with various diseases, including carcinomatosis, tuberculosis, leukemia, and polycythemia vera.

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agnogenic

of unknown origin.

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agnogenic myeloid metaplasia

see myeloid metaplasia.

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agnogenic myeloid metaplasia

Myelofibrosis with myeloid metaplasia Hematology A chronic progressive condition–panmyelosis and variable BM fibrosis, massive splenomegaly 2º to extramedullary hematopoiesis and leukoerythroblastic anemia with dysmorphic RBCs, circulating normoblasts, immature WBCs, atypical platelets Clinical Pts are often > age 50, insidious weight loss, anemia, abdominal discomfort due to splenomegaly, often with hepatomegaly; 80% have nonspecific chromosome defects Diagnosis BM Bx Management No specific therapy; packed RBCs for anemia, androgens may ↓ transfusion requirements, but are poorly tolerated in ♀; recombinant erythropoietin Prognosis Survival ± 5 yrs, often → acute leukemia. See Pseudonym syndrome.