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Chronic basophilic leukemia: a distinct clinico-pathologic entity?

Animesh D Pardanani1, William G Morice, James D Hoyer

  • 1Divisions of Hematology and Internal Medicine, Hematopathology, Mayo Clinic, Rochester, MN 55905, USA.

European Journal of Haematology
|June 13, 2003
PubMed
Summary
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This study defines a rare chronic basophilic leukemia, a myeloproliferative disorder with prominent basophilia. The condition can be aggressive, potentially transforming into acute myeloid leukemia.

Area of Science:

  • Hematology
  • Oncology

Background:

  • Myeloproliferative disorders are a group of clonal hematopoietic stem cell diseases.
  • Chronic basophilia is a rare finding in myeloproliferative disorders, particularly without the Philadelphia chromosome.

Observation:

  • Four cases of Philadelphia chromosome-negative myeloproliferative disorders with prominent chronic basophilia were identified.
  • Patients presented with mediator-release syndromes, pituitary dysfunction, or bone marrow abnormalities.

Findings:

  • Bone marrow examination revealed trilineage hyperplasia with basophilia, eosinophilia, and dysplastic megakaryocytic hyperplasia.
  • Atypical mast cell patterns were observed in two cases.
  • One patient transformed to acute myeloid leukemia, while another achieved long-term remission after stem cell transplantation.

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Implications:

  • These findings suggest a distinct clinico-pathologic entity, potentially termed chronic basophilic leukemia.
  • This aggressive disorder warrants further investigation due to its potential for transformation into acute leukemia.