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Hairy cell leukemia: clinical features and therapeutic advances

Cancer and Metastasis Reviews volume 6, pages 283–300 (1987)Cite this article

Abstract

Hairy cell leukemia (HCL) is a rare chronic lymphoproliferative disorder which has been extensively studied over the past decade. Much has been learned regarding the diagnosis, natural history, biology, and treatment of this unique neoplasm. The disease most commonly affects middle aged men and characteristic clinical features include splenomegaly, cytopenias, and usually the presence in the peripheral blood of distinctive thairy cells with irregular cytoplasmic projections. Diagnosis can usually be confirmed by bone marrow biopsy. Although the natural history can be extremely variable among patients, complications are usually referable to the cytopenias, with anemia and infection being most frequent. In addition to pyogenic infections, patients are susceptible to unusual organisms including atypical mycobacterium, legionella, and fungi. The requirement of red blood cell transfusion, severe granulocytopenia or thrombocytopenia, frequent infections, or painful splenomegaly are all indications for treatment. Splenectomy is the standard initial treatment of choice. However, in the past few years there have been exciting major advances in the therapeutic modalities for HCL. Recombinant alfa-interferon is highly effective, with beneficial responses occurring in close to 90% of patients. The Food and Drug Administration has recently approved the use the interferon for HCL. This represents the first time a biological response modifier has been approved for the treatment of human disease. In addition, preliminary results with the adenosine deaminase inhibitor, 2′ deoxycoformycin (def), have been encouraging. Further clinical trials are required in order to determine the optimal sequential treatment strategy for HCL. The exact mechanisms of action of both interferon and def in HCL remain to be elucidated. A better understanding of the unusual features of the hairy cell and the underlying biological effect of these two agents in HCL may have important applications in other hamatologic and non-hematologic malignancies.

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Affiliations

  1. The Department of Medicine, Joint Section of Hematology/Oncology, The University of Chicago Medical Center, Chicago, Illinois, USA

    Barry C. Lembersky & Harvey M. Golomb

  2. Michael Reese Medical Center, Chicago, Illinois, USA

    Barry C. Lembersky & Harvey M. Golomb

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  1. Barry C. Lembersky
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Lembersky, B.C., Golomb, H.M. Hairy cell leukemia: clinical features and therapeutic advances. Cancer Metast Rev 6, 283–300 (1987). https://doi.org/10.1007/BF00144268

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Key words

  • hairy cell leukemia
  • clinical features
  • treatment
  • interferon
  • 2′-deoxyformycin