Primary myelodysplastic syndrome: clinical spectrum of 53 cases.
Fifty three (45 males, 8 females) patients with primary meylodysplastic syndrome were seen between January 1990 and June, 1996. Fifteen (28%) patients had refractory anaemia (RA), 9 (17%) refractory anaemia with ring sideroblast (RARS), 21 (40%) refractory anaemia with excess blasts (RAEB), 5 (9%) refractory anaemia with excess blasts in transformation (REABt) and 3 (6%) had chronic myelomonocytic leukemia (CMML). The mean age for the whole cohort was 59 years. Patients with RAEB and RAEBt were significantly younger than other FAB types with a mean age of 53.5 and 45 years respectively. Among the FAB types RAEB appeared to be over represented. Symptomatic anaemia (66% cases) was the major cause to seek medical attention. The commonest laboratory findings was anaemia; Hb < 8 g/dl in 31 (59%) patients. Only two patients had Hb > 12 g/dl at presentation. Twenty four (45%) patients had normocytic anaemia, mainly in RAEB group (61%). Macrocytosis was a dominant finding in patients with RA (53%) and RARS (53%). Bicytopenia (72%) was a more common finding than pancytopenia (8%). Bone marrow was normocellular in 32 (60%) patients and hypoplastic in 11 (21%). Dyserythropoiesis predominantly affected RA (80%), RARS (55%) and RAEB (43%) groups. Bilineage dysplasia (21%) was commoner than trilineage dysplasia (19%). Increased bone marrow fibrosis was seen in about half of the available trephines, mainly in RAEB patients. Median survival of patients was ten months with a follow up duration of 2-55 months. Four patients transformed to acute leukemia (M1 or M2) and died subsequently. However, infection was major complication and cause of death (10 cases). The preponderance of younger people to acquire the disease (especially the RAEB and RAEBt variants), the emergence of RAEB as the major group of MDS and increased prevalence of hypoplastic MDS point towards non-therapeutic genotoxin (s) in the causation of disease. Shortened survival and low rate of acute transformation points that patients did not withstand cytopenias and died earlier.
Irfan
M
M
Department of Pathology, Aga Khan University Hospital, Karachi.
Kakepoto
G N
GN
Khursheed
M
M
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000097:blood / Q000175:diagnosis*; D012189:Retrospective Studies