Dysmyelopoietic syndrome(DMPS) is a primary hematologic disorder characterized by
ineffective hematopoiesis that the bone marrow is usually cellular despite associated
pancytopenia in the peripheral blood and aberrent cellular development result in abnormal
morphologic features involving nucleus and cytoplasm. In some cases, DMPS progress to
acute myeloid leukemia. Because the natural course of DMPS is different from AML, it
is important to differentiate DMPS from AML in management and prognosis. In 1982,
FAB (French-American-British) group suggested classification and features of DMPS in
detail. Here, we present 16 cases of DMPS we has experienced from June, 1981 to July,
1984.
1) The peak age incidence was in the 5th decade and male to female ratio was 2.2 :
1.
2) The most common symptoms were dyspnea on exertion(50.0%) and generalized
weakness(37.5%); and the most common sign was pallor (87.5%) followed by
hepatosplenomegaly (50.0%), bleeding manifestation (43.8%), lymphadenopathy(25.0%),
fever(18.8%) and sternal tenderness(18.8%) in order.
3) The abnormal hematologic findings were anemia(100%), leucopenia (62.5%) and
thrombocytopenia(87.5%). The most common subtype was RAEB(43.8%), followed by
RAEB in transformation (31.3%) and RA(25.0%).
4) Ten out of sixteen cases(62.5%) have experienced infectious complication and six
cases (37.5%) have experienced bleeding complication during hospital period.
5) Three out of 7 cases(42.9%), were converted to AML within 8 months and six
cases expired due to sepsis (five cases) and bleeding (one case).
|