Hairy cell leukemia: diagnostic pathology

Hematol Oncol Clin North Am. 2006 Oct;20(5):1023-49. doi: 10.1016/j.hoc.2006.06.010.


The pathology of HCL has been reviewed with a focus on the diagnostic hematopathology of this rare, but fascinating, disease. The discrimination of HCL from other B-cell lymphoproliferations, particularly HCL-V and SMZL, has been emphasized. The unique responsiveness of HCL to 2-CdA and other chemotherapeutic agents makes this distinction critical. Fortunately, HCL has consistent cytologic, histologic, cytochemical, and immunologic features that make classification reliable and reproducible. Less straightforward is the differential diagnosis of SMZL and HCL-V, problematic because of the rarity of both disorders, lack of discriminating evidence-based criteria, and perhaps a biologic kinship between these two disorders that share many clinical and pathologic features. Fortunately, this is not a clinically critical distinction.

Publication types

  • Review

MeSH terms

  • Antigens, Neoplasm / blood
  • Antigens, Neoplasm / immunology*
  • Biomarkers, Tumor / blood
  • Biomarkers, Tumor / immunology*
  • Bone Marrow / immunology
  • Bone Marrow / metabolism
  • Bone Marrow / pathology
  • Diagnosis, Differential
  • Flow Cytometry / methods
  • Humans
  • Leukemia, Hairy Cell / blood
  • Leukemia, Hairy Cell / immunology*
  • Leukemia, Hairy Cell / pathology*
  • Lymph Nodes / immunology
  • Lymph Nodes / metabolism
  • Lymph Nodes / pathology
  • Lymphoma, B-Cell / blood
  • Lymphoma, B-Cell / immunology
  • Lymphoma, B-Cell / pathology
  • Lymphoma, Non-Hodgkin / blood
  • Lymphoma, Non-Hodgkin / immunology
  • Lymphoma, Non-Hodgkin / pathology
  • Spleen / immunology
  • Spleen / metabolism
  • Spleen / pathology


  • Antigens, Neoplasm
  • Biomarkers, Tumor