Acute lymphoid leukemia following polycythemia vera
Por:
Camós-Guijosa M, Cervantes F, Montoto S, Hernández-Boluda JC, Villamor N and Montserrat E
Publicada:
1 ene 1999
Resumen:
The tendency to evolve into acute leukemia is a well-known
characteristic of polycythemia vera (PV), which is shared with the
remaining chronic myeloproliferative disorders and increases after the
administration of cytotoxic agents. Acute transformation is usually of
myeloid phenotype, whereas acute lymphoid leukemia (ALL) following PV is
seldom observed. A 63-year-old woman is described who developed ALL at 6
years from the initial diagnosis of PV, for which she had received
radioactive phosphorus and hydroxyurea. The ALL was of B-cell type,
corresponding to the L-3 subtype of the FAB classification. Despite the
administration of combination chemotherapy the patient died shortly
after the diagnosis of acute leukemia. The present case adds to seven
previously described patients with the above association, all of whom
had received cytotoxic therapy for PV. Median interval from PV to ALL
diagnosis was 10 years, and there was a predominance of the B-cell
phenotype. The prognosis was poor since all but one of the patients had
a short survival after ALL diagnosis. The possible etiological and
pathogenetic link between PV and the subsequent ALL is discussed.
Filiaciones:
Camós-Guijosa M:
Postgraduate School of Hematology, Farreras Valenti, Hematology Department, IDIBAPS, Hospital Clínic, Barcelona, Spain
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