Isochromosome 17q in Ph1-negative leukemia: a clinical, cytogenetic, and
molecular study [see comments]
R Becher, F Carbonell and CR Bartram
Innere Universitats-und Poliklinik (Westdeutsches Tumorzentrum), Essen,
FRG.
We report on eight patients who were 35 to 77 years old with an
isochromosome 17q as the sole structural chromosomal anomaly. Additional
numerical chromosomal changes were a trisomy 8 or 17 in two cases each and
a trisomy 19 in one case. Five patients had myelodysplastic syndrome (MDS)
diagnosed according to the FAB nomenclature as chronic myelomonocytic
leukemia (CMML) in two cases, refractory anemia with excess of blasts in
transformation (RAEBt) in two cases, and refractory anemia with excess of
blasts (RAEB) in one case. One patient suffered from a myeloproliferative
disorder (MPS). All cases progressed to acute nonlymphocytic leukemia
(ANLL) type M1, M2, or M4 in a period of 2 to 30 months after initial
diagnosis, except one patient with RAEBt who died within 2 months. Two
patients presented with ANLL-M2 at time of diagnosis. Treatment during the
chronic phase of disease consisted of mild cytoreduction and/or
substitution of platelets or red blood cells. One patient with CMML
received an allogeneic bone marrow graft and relapsed after 33 months with
ANLL-M1. Treatment results for overt leukemia were poor, and survival was
short, lasting from 1 to 4 months. Overall survival was 1 to 37 months
(median duration, 6.5 months). Molecular studies in two cases revealed
neither a BCR rearrangement nor a translocation of the ABL protooncogene,
as observed in Ph1-positive chronic myeloid leukemia (CML). Thus, an i(17q)
anomaly seems to identify a distinct subgroup of mostly myelodysplastic
and, less frequently, myeloproliferative disorders that progress rapidly to
ANLL, respond poorly to chemotherapy, and are associated with short
survival after transformation.
Volume 75,
Issue 8,
pp. 1679-1683,
04/15/1990
Copyright © 1990 by The American Society of Hematology
