Hairy Cell Leukemia Clinical Presentation

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

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 scleroderma, polymyositis, polyarteritis nodosa, erythematous maculopapules, and 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, 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

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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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