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Hairy Cell Leukemia Clinical Presentation

  • Author: Emmanuel C Besa, MD; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
 
Updated: Apr 28, 2015
 

History

The most common symptoms and presenting complaints in hairy cell leukemia are weakness and fatigue due to anemia. Approximately one third of patients have bleeding from thrombocytopenia, and another one third have fever and infections from neutropenia.

Symptoms related to organ infiltration of the reticuloendothelial system may occur. Abdominal discomfort from an enlarged spleen is present in one quarter of patients.

Some patients may present with weight loss, fever, and night sweats, similar to other lymphoproliferative disorders.

Hairy cell leukemia is associated with gram-positive and gram-negative bacterial infections, as well as atypical mycobacterial and invasive fungal infections. Other opportunistic infections, such as Legionella, toxoplasmosis, and listeriosis, have been reported.

Hairy cell leukemia is associated with other systemic immunologic disorders including the following:

  • Scleroderma
  • Polymyositis
  • Polyarteritis nodosa
  • Erythematous maculopapules
  • Pyoderma gangrenosum

Other uncommon conditions may be associated with hairy cell leukemia, such as acquired factor VIII antibodies, paraproteinemia, and systemic mast cell disease.

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Physical

Splenomegaly is the most common physical finding in virtually every patient with hairy cell leukemia, and it is massive in more than 80% of patients.

Hepatomegaly with mild liver function abnormalities is found in 20% of cases, and lymphadenopathy is found in 10%.

A low-grade fever is part of the disease, but it may be due to an infection from the resulting neutropenia. In more than half of the cases, it is from a gram-negative organism. Atypical mycobacterial infections are common. Disseminated fungal infections and Pneumocystis carinii may occur in some cases of hairy cell leukemia.

Peripheral lymphadenopathy is uncommon, with fewer than 10% of patients presenting with peripheral nodes larger than 2 cm in diameter. However, internal adenopathy may develop after a prolonged disease course and was found in 75% of patients at autopsy.

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Causes

Hairy cell leukemia is due to proliferation of a clonal malignant B cell that infiltrates the reticuloendothelial cells, particularly the bone marrow, resulting in bone marrow failure.

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Contributor Information and Disclosures
Author

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, 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 Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Koyamangalath Krishnan, MD, FRCP, FACP Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine, 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, Royal College of Physicians

Disclosure: Nothing to disclose.

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

  2. Cannon T, Mobarek D, Wegge J, Tabbara IA. Hairy cell leukemia: current concepts. Cancer Invest. 2008 Oct. 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. 2011 Nov 9. [Medline].

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

  5. Tiacci E, Trifonov V, Schiavoni G, Holmes A, Kern W, Martelli MP. BRAF mutations in hairy-cell leukemia. N Engl J Med. 2011 Jun 16. 364(24):2305-15. [Medline].

  6. 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. 2008 Nov 18. epub ahead of print. [Medline].

  7. Matutes E. Immunophenotyping and differential diagnosis of hairy cell leukemia. Hematol Oncol Clin North Am. 2006 Oct. 20(5):1051-63. [Medline].

  8. 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. 2011 Oct. 136(4):625-30. [Medline].

  9. 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. 2011 Sep. 136(3):390-9. [Medline].

  10. Robak T. Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment. Cancer Treat Rev. 2011 Feb. 37(1):3-10. [Medline].

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

  12. Ng JP, Nolan B, Chan-Lam D, Coup AJ, McKenna D. Successful treatment of aplastic variant of hairy-cell leukaemia with deoxycoformycin. Hematology. 2002 Aug. 7(4):259-62. [Medline].

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

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

  15. Grever MR. How I treat hairy cell leukemia. Blood. 2010 Jan 7. 115(1):21-8. [Medline].

  16. Else M, Dearden CE, Matutes E, Garcia-Talavera J, Rohatiner AZ, Johnson SA. Long-term follow-up of 233 patients with hairy cell leukaemia, treated initially with pentostatin or cladribine, at a median of 16 years from diagnosis. Br J Haematol. 2009 Jun. 145(6):733-40. [Medline].

  17. 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. 1990 Apr 19. 322(16):1117-21. [Medline].

  18. Jehn U, Bartl R, Dietzfelbinger H, Haferlach T, Heinemann V. An update: 12-year follow-up of patients with hairy cell leukemia following treatment with 2-chlorodeoxyadenosine. Leukemia. 2004 Sep. 18(9):1476-81. [Medline].

  19. Habermann TM, Andersen JW, Cassileth PA, Bennett JM, Oken MM. Sequential administration of recombinant interferon alpha and deoxycoformycin in the treatment of hairy cell leukaemia. Br J Haematol. 1992 Apr. 80(4):466-71. [Medline].

  20. Saven A, Burian C, Adusumalli J, Koziol JA. Filgrastim for cladribine-induced neutropenic fever in patients with hairy cell leukemia. Blood. 1999 Apr 15. 93(8):2471-7. [Medline].

  21. 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. 1990 Apr 19. 322(16):1117-21. [Medline].

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

  23. 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. 2011 Oct 31. [Medline].

  24. 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. 2005 Jul 1. 106(1):241-6. [Medline]. [Full Text].

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

  26. 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. 2011 Jun. 52 Suppl 2:82-6. [Medline].

  27. 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. 2000 Nov 1. 96(9):2981-6. [Medline]. [Full Text].

  28. Kreitman RJ, Fitzgerald DJ, Pastan I. Approach to the patient after relapse of hairy cell leukemia. Leuk Lymphoma. 2009 Oct. 50 Suppl 1:32-7. [Medline].

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

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

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

  32. 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. 2008 Dec. 19(10):1147-60. [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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