HEMATOLOGIC, CLINICAL, AND CYTOGENETIC ANALYSIS IN 109 PATIENTS WITH PRIMARY MYELODYSPLASTIC-SYNDROME - PROGNOSTIC-SIGNIFICANCE OF MORPHOLOGY AND CHROMOSOME FINDINGS

One hundred and nine patients with primary myelodysplastic syndrome (M DS) were classified according to the French-American-British (FAB) cri teria: 27 refractory anemia (RA, 25%), 26 RA with ringed sideroblasts (RARS, 24%), 16 RA with excess of blasts (RAEB, 15%), 10 RAEB in trans formation (RAEB-t, 9%), 25 chronic myelomonocytic leukemia (CMMoL, 23% ), and five unclassifiable MDS (4%). Forty-three were women and 66 wer e men (sex ratio 2:3). Age ranged from 30-92 years (mean 69 years) wit h nine patients aged less than 50 years (8%). A cytogenetic result was obtained in all cases. At initial study, a chromosome defect was obse rved in 56% of patients. Rates of abnormality depended on FAB subtype: 52% in RA, 100% in RA 5q-, 50% in RARS, 56% in RAEB, 70% in RAEB-t an d 44% in CMMoL. The most frequent single defects were del(5q), -7/del/ (7q), del(20q), Y loss, and +8. Except for the 5q syndrome entity, spe cific chromosome defects were not associated with particular FAB subty pes. Bone marrow (BM) insufficiency (22%) and leukemic transformation (21%) were the most important causes of death. The rate of leukemic tr ansformation increased with the number of dysplastic BM cell lineages and was also associated with karyotype complexity and the proportion o f abnormal/normal metaphases. The longest median survivals were observ ed in BARS (142 months) and RA/RA5q- (91 months) types. Median surviva ls decreased with increasing Bournemouth score values. Patients with t hree abnormal cell lineages had a median survival shorter than those w ith one or two abnormal lineages. Similarly patients with complex defe cts had shorter survival than those with single or double defects or a normal karyotype. There was no statistically significant difference b etween survival of NN (normal), AN (abnormal/normal), and AA patients or between survival of patients with del(5q), -7/del(7q), +8 or del(20 q).