Clinico-hematological characteristics of acute transformation of chronic myeloproliferative syndromes

Abstract:

Background The aim of the present study was to analyze the blastic transformation occurring during chronic myeloproliferative diseases (CMPD) and to establish the differences between them. Methods The clinical and hematological characteristics of 54 patients in blastic crisis (BC) of a CMPD were analyzed: 40 chronic myelogenous leukemias (CML), 9 idiopathic myelofibroses (IMF), 4 polycythemia vera (PV), and one essential thrombocythemia (ET). The results were analyzed by the BMDP statistical program. Results The BC of CML appeared in younger patients (p less than 0.05). Only in this group did some patients achieve complete remission. Moreover, in this BC a greater incidence of visceromegalies and leukocytoses were observed. The BC of the IMF patients led to marked anemia (p less than 0.01) and bone marrow infiltration (p less than 0.05); these leukemias were more frequent in males and began with lymphadenopathies and visceromegalies. The transformations of PV were preceded by a longer chronic phase and had a lower incidence of visceromegalies and a nearly normal hemoglobin count value. The patient with acute leukemia secondary to ET did not display visceromegalies but did have anemia, leukopenia and a normal platelet count. None of the four groups responded to therapy, and their survival was short. Conclusions Blast transformations of different myeloproliferative disorders have their own idiosynchratic clinical and hematological characteristics, some of these may be related to the chronic phase of disease.

Año de publicación:

1991

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