Hairy Cell Leukemia 

  • Author: Emmanuel C Besa, MD; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP   more...
 
Updated: Feb 14, 2012
 

Background

Hairy cell leukemia (HCL) is a chronic lymphoid leukemia that was originally described in 1958 by Bouroncle and colleagues.[1, 2] Hairy cell leukemia is a B-cell disease, and the abnormal cell has hairlike cytoplasmic projections on its surface.

Hairy cell leukemia is recognized as a clonal B-cell malignancy as identified by immunoglobulin gene rearrangements that result in a phenotype B-cell expression of surface antigens, which reflect the differentiation between the immature B-cell of chronic lymphocytic leukemia and the plasma cell of multiple myeloma.

For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center. Also, see eMedicine's patient education article Leukemia.

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Pathophysiology

The abnormal cell in hairy cell leukemia is a clonal B-cell lymphocyte (see image below). This cell infiltrates the patient's reticuloendothelial system and interferes with bone marrow function, resulting in bone marrow failure or pancytopenia.[3, 4] The hairy cell also infiltrates the liver and spleen, resulting in organomegaly.

Blood film at × 400 magnification. This image demoBlood film at × 400 magnification. This image demonstrates a lymphocytosis and an absence of any other type of blood cell (pancytopenia). The characteristic cytoplasmic projections are already visible. Photographed by U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.

The etiology of hairy cell leukemia has not been determined, although some investigators suggest that exposures to benzene, organophosphorus insecticides, or other solvents may be related to disease development. This hypothesis has not been confirmed by other reports, although a French study that evaluated occupational exposure to pesticides and lymphoid neoplasms among men appears to support the hypothesis that occupational pesticide exposures may not only be involved in hairy cell leukemia, Hodgkin lymphoma, and multiple myeloma, but also may play a role in non-Hodgkin lymphoma.[5] Further research is needed.

Exposure to radiation, agricultural chemicals, and wood dust, and a previous history of infectious mononucleosis have been suggested as etiologic associations in previous reports.

Overexpression of cyclin D1 protein, an important cell-cycle regulator, has been observed in hairy cell leukemia and may play a role in the molecular pathogenesis of the disease.

Accumulation of hairy cells in the bone marrow, liver, and spleen, with very little lymph node involvement, is characteristic of hairy cell leukemia. This pattern probably results from the expression of the integrin receptor alpha4-beta1 by the hairy cells and the interaction of the receptor with the vascular adhesion molecule-1 (VCAM-1) found in splenic and hepatic endothelia, bone marrow, and splenic stroma.

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Epidemiology

Frequency

United States

Hairy cell leukemia is relatively uncommon and accounts for 2% of all leukemia cases, which is about 600-800 new patients diagnosed each year.[2]

International

Some geographic variations have been observed with hairy cell leukemia, such as an extremely low incidence in Japan.

Mortality/Morbidity

  • Symptoms of pancytopenia in hairy cell leukemia are related to anemia (fatigue and weakness), thrombocytopenia (bleeding or easy bruising), and neutropenia (infections).
  • Abdominal discomfort is a common symptom, resulting from hepatosplenomegaly. It is usually controlled with new effective drug therapy, although late recurrences may occur. Recurrences are usually responsive to medications.

Race

  • This disease is observed more commonly in whites. It has been reported to occur in Ashkenazi Jewish males, and it is rare in Japan and in those of African descent.[2]

Sex

  • Hairy cell leukemia is observed mostly in males, with a male-to-female ratio of 4:1 to 5:1.[2]

Age

  • Hairy cell leukemia occurs predominantly in middle-aged men, with a median age of 52 years.[2]
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Contributor Information and Disclosures
Author

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Coauthor(s)

Ulrich Josef Woermann, MD  Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland

Disclosure: Nothing to disclose.

Specialty Editor Board

Rodger L Bick†, MD, PhD, FACP  Former Clinical Professor of Medicine, University of Texas Southwestern Medical Center; Former Director, Dallas and Pacific Thrombosis Hemostasis and Vascular Medicine Clinical Center

Rodger L Bick†, MD, PhD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Blood Banks, American Cancer Society, American College of Angiology, American College of Physicians, American Geriatrics Society, American Heart Association, American Medical Association, American Society for Clinical Pathology, American Society of Hematology, Association of Clinical Scientists, California Medical Association, California Thoracic Society, International College of Angiology, International Society of Hematology, International Society on Thrombosis and Haemostasis, New York Academy of Sciences, and Southwest Oncology Group

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Troy H Guthrie, Jr, MD  Director of Cancer Institute, Baptist Medical Center

Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Koyamangalath Krishnan, MD, FRCP, FACP  Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, James H Quillen College of Medicine at East Tennessee State University

Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, and Royal College of Physicians

Disclosure: Nothing to disclose.

References

  1. Bouroncle BA, Wiseman BK, Doan CA. Leukemic reticuloendotheliosis. Blood. Jul 1958;13(7):609-30. [Medline]. [Full Text].

  2. Cannon T, Mobarek D, Wegge J, Tabbara IA. Hairy cell leukemia: current concepts. Cancer Invest. Oct 2008;26(8):860-5. [Medline].

  3. Arcaini L, Zibellini S, Boveri E, Riboni R, Rattotti S, Varettoni M, et al. The BRAF V600E mutation in hairy cell leukemia and other mature B-cell neoplasms. Blood. Nov 9 2011;[Medline].

  4. Zinzani PL, Magagnoli M, Bendandi M, et al. Long-term follow-up of hairy cell leukemia patients treated with 2-chlorodeoxyadenosine. Haematologica. Sep 2000;85(9):922-5. [Medline]. [Full Text].

  5. Orsi L, Delabre L, Monnereau A, et al. Occupational exposure to pesticides and lymphoid neoplasms among men: results of a French case-control study. Occup Environ Med. Nov 18 2008;epub ahead of print. [Medline].

  6. Venkataraman G, Aguhar C, Kreitman RJ, Yuan CM, Stetler-Stevenson M. Characteristic CD103 and CD123 expression pattern defines hairy cell leukemia: usefulness of CD123 and CD103 in the diagnosis of mature B-cell lymphoproliferative disorders. Am J Clin Pathol. Oct 2011;136(4):625-30. [Medline].

  7. Sherman MJ, Hanson CA, Hoyer JD. An assessment of the usefulness of immunohistochemical stains in the diagnosis of hairy cell leukemia. Am J Clin Pathol. Sep 2011;136(3):390-9. [Medline].

  8. Katayama I. Bone marrow in hairy cell leukemia. Hematol Oncol Clin North Am. Dec 1988;2(4):585-602. [Medline].

  9. Arcaini L, Zibellini S, Boveri E, et al. The BRAF V600E mutation in hairy cell leukemia and other mature B-cell neoplasms. Blood. Jan 5 2012;119(1):188-91. [Medline].

  10. Tiacci E, Schiavoni G, Forconi F, et al. Simple genetic diagnosis of hairy cell leukemia by sensitive detection of the BRAF-V600E mutation. Blood. Jan 5 2012;119(1):192-5. [Medline].

  11. Piro LD, Carrera CJ, Carson DA, Beutler E. Lasting remissions in hairy-cell leukemia induced by a single infusion of 2-chlorodeoxyadenosine. N Engl J Med. Apr 19 1990;322(16):1117-21. [Medline].

  12. Goodman GR, Burian C, Koziol JA, Saven A. Extended follow-up of patients with hairy cell leukemia after treatment with cladribine. J Clin Oncol. Mar 1 2003;21(5):891-6. [Medline]. [Full Text].

  13. Ganzel C, Gatt ME, Maly A, Ben-Yehuda D, Goldschmidt N. High incidence of skin rash in patients with hairy cell leukemia treated with cladribine. Leuk Lymphoma. Oct 31 2011;[Medline].

  14. Chadha P, Rademaker AW, Mendiratta P, et al. Treatment of hairy cell leukemia with 2-chlorodeoxyadenosine (2-CdA): long-term follow-up of the Northwestern University experience. Blood. Jul 1 2005;106(1):241-6. [Medline]. [Full Text].

  15. Ravandi F, Jorgensen JL, O'Brien SM, et al. Eradication of minimal residual disease in hairy cell leukemia. Blood. Jun 15 2006;107(12):4658-62. [Medline]. [Full Text].

  16. Kreitman RJ, Arons E, Stetler-Stevenson M, Fitzgerald DJ, Wilson WH, Pastan I. Recombinant immunotoxins and other therapies for relapsed/refractory hairy cell leukemia. Leuk Lymphoma. Jun 2011;52 Suppl 2:82-6. [Medline].

  17. Flinn IW, Kopecky KJ, Foucar MK, et al. Long-term follow-up of remission duration, mortality, and second malignancies in hairy cell leukemia patients treated with pentostatin. Blood. Nov 1 2000;96(9):2981-6. [Medline]. [Full Text].

  18. Au WY, Klasa RJ, Gallagher R, et al. Second malignancies in patients with hairy cell leukemia in british columbia: a 20-year experience. Blood. Aug 15 1998;92(4):1160-4. [Medline]. [Full Text].

  19. Kurzrock R, Strom SS, Estey E, et al. Second cancer risk in hairy cell leukemia: analysis of 350 patients. J Clin Oncol. May 1997;15(5):1803-10. [Medline].

  20. Forconi F. Hairy cell leukaemia: biological and clinical overview from immunogenetic insights. Hematol Oncol. Jun 2011;29(2):55-66. [Medline].

  21. Glaspy JA, Baldwin GC, Robertson PA, et al. Therapy for neutropenia in hairy cell leukemia with recombinant human granulocyte colony-stimulating factor. Ann Intern Med. Nov 15 1988;109(10):789-95. [Medline].

  22. Monnereau A, Orsi L, Troussard X, Berthou C, et al. Cigarette smoking, alcohol drinking, and risk of lymphoid neoplasms: results of a French case-control study. Cancer Causes Control. Dec 2008;19(10):1147-60. [Medline].

  23. Ratain MJ, Golomb HM, Vardiman JW, et al. Relapse after interferon alfa-2b therapy for hairy-cell leukemia: analysis of prognostic variables. J Clin Oncol. Nov 1988;6(11):1714-21. [Medline].

 
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Blood film at × 400 magnification. This image demonstrates a lymphocytosis and an absence of any other type of blood cell (pancytopenia). The characteristic cytoplasmic projections are already visible. Photographed by U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
Blood film at × 1000 magnification. This image demonstrates lymphocytes with characteristic cytoplasmic projections. Photographed by U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
Blood film at × 1000 magnification. This image demonstrates tartrate-resistant acid phosphatase (TRAP) activity of lymphocytes. Photographed by U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
 
 
 
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