Hematological features of patients with myelodysplastic syndromes associated with a chromosome 5q deletion.
The hematological and clinical features of 26 patients with myelodysplasia and a chromosome 5q deletion in the bone marrow are presented. We have examined the relationship of French-American-British Co-operative Group (FAB) 1982 classification and bone marrow karyotype at diagnosis with patient outcome and the presence or absence of the classical features of the 5q-syndrome. Those patients classified as refractory anemia (RA) with no additional karyotypic abnormalities have the typical features of the 5q-syndrome and a good prognosis. None of the patients in this group transformed to acute leukemia during the period of follow-up. Patients with either refractory anemia and excess blasts (RAEB) or additional karyotypic abnormalities show many of the hematologic features of the 5q-syndrome but do not share the good prognosis. We conclude that the 5q-syndrome may be best defined as primary MDS of the FAB type RA with a 5q deletion as the sole karyotypic abnormality. This simple definition will distinguish patients with a good prognosis and all the classical features of the 5q-syndrome.
Lewis
S
S
Haematology Department, John Radcliffe Hospital, Oxford, United Kingdom.
Oscier
D
D
Boultwood
J
J
Ross
F
F
Fitchett
M
M
Rack
K
K
Abrahamson
G
G
Buckle
V
V
Wainscoat
J S
JS
D000208:Acute Disease; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000753:Anemia, Refractory / Q000235:genetics; D000754:Anemia, Refractory, with Excess of Blasts / Q000235:genetics; D001853:Bone Marrow / Q000473:pathology; D002895:Chromosomes, Human, Pair 5; D005260:Female; D017353:Gene Deletion; D006801:Humans; D007621:Karyotyping; D007938:Leukemia / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000145:classification / Q000235:genetics* / Q000473:pathology; D011379:Prognosis