Chronic basophilic leukemia: A distinct clinico-pathologic entity?

Research output: Contribution to journalArticle

18 Scopus citations

Abstract

Objective: We sought to better define a group of rare and poorly understood myeloproliferative disorders-that are characterized by prominent chronic basophilia in the absence of the Philadelphia chromosome (Ph) or its molecular equivalent. Methods: We screened our institution's electronic database from 1975 onwards, and identified four such cases. Clinical data and bone marrow pathology were carefully reviewed for these patients. Results: Two patients had prominent manifestations of basophil mediator-release and another presented with pituitary dysfunction. Bone marrow examination uniformly revealed trilineage hyperplasia with basophilia and eosinophilia, dysplastic megakaryocytic hyperplasia, and the absence of megakaryocyte clustering. An abnormal pattern of atypical mast cells was noted in two cases. While disease palliation was effectively achieved with hydroxyurea for one patient, transformation to acute myeloid leukemia was eventually observed in this case. Another patient has achieved long-term disease-free survival after undergoing allogeneic stem cell transplantation. Conclusions: Our observations reveal a striking pathologic similarity among all four cases, and suggest this disease, which may be aggressive with the potential to transform into acute leukemia, to possibly represent a distinct clinico-pathologic entity (chronic basophilic leukemia).

Original languageEnglish (US)
Pages (from-to)18-22
Number of pages5
JournalEuropean Journal of Haematology
Volume71
Issue number1
DOIs
StatePublished - Jul 1 2003

Keywords

  • Basophilia
  • Leukemia

ASJC Scopus subject areas

  • Hematology

Fingerprint Dive into the research topics of 'Chronic basophilic leukemia: A distinct clinico-pathologic entity?'. Together they form a unique fingerprint.

  • Cite this