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The 5q- syndrome: a single-institution study of 43 consecutive patients
P Mathew, A Tefferi, GW Dewald, SL Goldberg, J Su, HC Hoagland and P Noel
Division of Hematology, Mayo Clinic, Rochester, MN 55901.
A favorable prognosis and a low rate of leukemic transformation has been
attributed to the 5q- syndrome, a myelodysplastic syndrome (MDS)
characterized by macrocytic anemia, hypolobulated micromegakaryocytic
hyperplasia, and an interstitial deletion of chromosome 5. We examined the
characteristics and outcome of 43 consecutive patients in our institution
strictly defined by morphologic criteria and a solitary 5q- cytogenetic
defect. The median age at diagnosis was 68 years, with a clear female
predominance (7:3). Eighty percent of the patients were red blood cell
transfusion-dependent at diagnosis and all untransfused patients had
macrocytic indexes. In contrast, significant neutropenia or
thrombocytopenia was rare. The French-American-British (FAB) class
distributions were RA (72%), RARS (7%), RAEB (16%), and RAEB-IT (5%). At a
median follow-up of 31 months, 56% of the patients survive, with a
projected median survival of 63 months. The incidence of acute leukemia was
16% and was uniformly fatal. Clinical hemosiderosis occurred in 28% of the
patients, resulting in two deaths. Neither survival nor the risk of
leukemic transformation was predictable from initial clinical parameters,
including FAB classification, Bournemouth score, and degree of aneuploidy.
The lack of significant neutropenia and thrombocytopenia seemed to account
for a very low incidence of infection and bleeding resulting in a prognosis
equal or superior to historical patients with MDS. Therapeutic endeavors,
including the use of corticosteroids, androgens, cis-retinoic acid,
pyridoxine, and danazol, were largely unsuccessful.
Volume 81,
Issue 4,
pp. 1040-1045,
02/15/1993
Copyright © 1993 by The American Society of Hematology
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