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Philadelphia-negative (Ph-) chronic myeloid leukemia (CML): comparison with
Ph+ CML and chronic myelomonocytic leukemia. The Groupe Francais de
Cytogenetique Hematologique
P Martiat, JL Michaux and J Rodhain
Department of Hematology, Cliniques Universitaires Saint-Luc, UCL,
Brussels, Belgium.
To better understand the Philadelphia-negative (Ph-) chronic myeloid
leukemia (CML) and its relationships with Philadelphia-positive (Ph+) CML
and chronic myelomonocytic leukemia (CMML), a study was undertaken by the
Groupe Francais de Cytogenetique Hematologique. Thirty-five Ph- CML
patients were investigated and compared with 55 chronic phase Ph+ CML and
100 CMML patients. There were 12 M-BCR positive (M-BCR+) and 23 M-BCR
negative (MBCR+) patients. No clinical or biologic differences were found
between Ph+ and Ph-, M-BCR+ patients. In the Ph- group, M- BCR+ and M-BCR-
patients differed significantly in age (47.7 +/- 6.6 v 67.0 +/- 6.1 years,
respectively; P = .001), leukocytosis (153.4 +/- 135.1 v 58.5 +/- 37.7
10(9)/L, P = .002), relative monocytosis (1.8% +/- 1.2% v 5.6% +/- 1.4%, P
= .048), absolute basophilia (8.5 +/- 9.7 v 0.9 +/- 1.5 10(9)/L, P = .001),
percentage of immature myeloid precursors (IMP) in peripheral blood (29.0%
+/- 9.5% v 15.3% +/- 8.1%, P = .001), and percentage of erythroblasts in
bone marrow (BM) (6.5% +/- 3.5% v 14.6% +/- 3.6%, P = .001). Karyotypic
abnormalities other than the Ph chromosome occurred in 0 of 12 M-BCR- at
diagnosis and 7 of 23 M-BCR- Ph- CML (P = .033). None of the 13
investigated BCR- patients had detectable BCR/ABL transcripts using
polymerase chain reaction (PCR) and none had an N-RAS mutation. Cytologic
findings showed a marked morphologic difference between M-BCR+ and M-BCR-
patients, especially in the monocytic lineage. Dysmyelopoietic features in
CMML and M-BCR- patients were very similar, and the differences were of
quantitative order only. Using four criteria (monocytosis, percentage of
IMP, basophilia, and percentage of erythroblasts in BM), patients could be
divided into typical and atypical CML and this classification correlated
well with molecular findings. We conclude that, while Ph-, M- BCR+, and Ph+
CML are identical diseases, Ph-, M-BCR- CML, and CMML have many
similarities and might be only different aspects of a same entity.
Volume 78,
Issue 1,
pp. 205-211,
07/01/1991
Copyright © 1991 by The American Society of Hematology

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