Experience with primary myelodysplastic syndromes in a university haematology unit: report of five cases.

Myelodysplastic syndromes apper to be a rare group of blood disorders in Malaysia. It usually affects the elderly and some of the cases were reported to evolve into acute leukemia. We describe our experience with five cases, seen and managed by us over a 5-year period. All patients receiving supportive therapy died with one of them definitely known to transform to acute leukemia. The patient who survives till the time of writing received low dose continuous cytosine arabinoside infusion. INTRODUCTION The myelodysplastic syndromes (MDS)' denote a group of blood disorders chracterised by peripheral blood cytopenia(s) in the presence of hypercellular bone marrow with features of ineffective haemopoiesis. The French-American-British (FAB)2 group classified them into five subtypes: Refractory Anaemia (RA); Refractory Anameia with Ring Sideroblasts (RARS); Refractory Anaemia with excess of Blasts (RAEB); Chronic Myelomonocytic Leukaemia (CMML); and RAEB in Transformation to Acute Myeloid Leukaemia (RAEB/Tr). This has generally been accepted and found to have useful prognostic implications. Various aspects of M DS have been reviewed recently". We describe our experience With five such cases. MATERIALS AND METHODS The clinico-pathologic features of five cases of primary myelodysplastic syndrome seen and managed by us between January 1981 and December 1985 were reviewed. We manage on an average about 50 new cases of malignant haematologic disorders every year. Blood count was performed by Coulter Sand Coulter thrombocounter. Wright's stain was used for peripheral blood films and May-Grunwald-Giemsa stain for bone marrow smears. Bone marrow biopsy findings and chromosome analysis were omitted in the review as not all cases had the date. The diagnosis was established according to the FAB classification. RESULTS The patient characteristics and clinical findings at presentation are summarised in Table I; and their haematologic features in Table 11. Prognostication of the cases was made using Multi's method". The results of "predicted median survival" by this method and actual survival of the patients are tabulated in Table Ill.