Transition of polycythemia vera to chronic neutrophilic leukemia

Citation
T. Higuchi et al., Transition of polycythemia vera to chronic neutrophilic leukemia, LEUK LYMPH, 33(1-2), 1999, pp. 203-206
Citations number
21
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Hematology,"Onconogenesis & Cancer Research
Journal title
LEUKEMIA & LYMPHOMA
ISSN journal
1042-8194 → ACNP
Volume
33
Issue
1-2
Year of publication
1999
Pages
203 - 206
Database
ISI
SICI code
1042-8194(199903)33:1-2<203:TOPVTC>2.0.ZU;2-V
Abstract
Two cases of polycythemia vera (PV) had transition to a hematological condi tion compatible with chronic neutrophilic leukemia (CNL) 17 and 8 years aft er diagnosis, respectively. One patient was treated with carboquone followe d by hydroxyurea (HU) and the other with HU during PV phase. On transition, both had neutrophilia with white blood cell count above 40,000/mu 1, eleva ted neutrophil alkaline phosphatase activity, splenomegaly, normal karyotyp e without bcr-abl rearrangement. Busulfan was temporally effective in contr olling the neutrophil count. However, one patient progressed to the so-call ed spent phase and the other subsequently had multiple transitions between PV and CNL. These cases may represent a form of uncommon evolution of PV an d support the contention that CNL is a type of myeloproliferative disorder and that at least some CNL cases have derangement at the hematopoietic stem cell level.