T. Duell et al., CHRONIC MYELOID-LEUKEMIA ASSOCIATED HYPEREOSINOPHILIC SYNDROME WITH ACLONAL T(4-7)(Q11-Q32), Cancer genetics and cytogenetics, 94(2), 1997, pp. 91-94
Patients with chronic myeloid leukemia (CML) show the Philadelphia (Ph
) translocation in more than 95% of the cases. The remaining cases, wi
thout the cytogenetic or molecular equivalent of the BCR-ABL rearrange
ment, are ''Philadelphia negative'' and may have alternate chromosomal
aberrations. Ph negative CML patients are known to have a poor progno
sis. We report on a young patient with a hypereosinophilic syndrome in
the presence of a clonal translocation t(4;7) with a peripheral leuko
cytosis, a severe thrombocytopenia, and anemia at first presentation,
who developed bone marrow changes typical of CML. bone marrow function
and hypereosinophilia improved only partially and temporarily under t
herapy. The patient died 10 months after diagnosis of diffuse leukemic
embolism and organ infiltration resulting in paraplegia. The case dem
onstrates that beside ''idiopathic'' hypereosinophilic syndromes (HES)
, a proportion of such patients suffer from eosinophilic leukemias. In
these cases, karyotype analysis may help to distinguish these states
by the identification of clonal chromosomal abnormalities. A karyotype
anomaly hitherto not reported can be added to the list of aberrations
in hypereosinophilic states associated with myeloproliferative proces
ses. (C) Elsevier Science Inc., 1997.