Hairy Cell Leukemia Medication

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

Medication Summary

Systemic therapy has changed rapidly in the past 10 years because of new biologic agents (eg, interferons) and new purine analogues. Also, the availability of recombinant human hematopoietic growth factors has improved supportive care during life-threatening infections in those with hairy cell leukemia.

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Purine Analogues

Class Summary

Gibbett and coworkers observed that patients with severe combined immunodeficiency (SCID) were deficient in the purine catabolic enzyme adenosine deaminase. This deficiency causes intracellular accumulation of deoxyadenosine triphosphate and results in lymphocytotoxicity. Purine analogues mimic this condition by irreversibly binding to adenosine deaminase or by fostering resistance to deamination in the purine salvage pathway.

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Cladribine or 2-chlorodeoxyadenosine (Leustatin)

 

Synthetic antineoplastic agent for continuous IV infusion. The enzyme deoxycytidine kinase phosphorylates this compound into active 5'-triphosphate derivative. This, in turn, breaks DNA strands, inhibits DNA synthesis, disrupts cell metabolism, and causes death to resting and dividing cells.

Most active among the purine analogues in the treatment of hairy cell leukemia. Has a 94% overall response and 84% complete response in hairy cell leukemia.

Patients whose disease does not respond to the initial regimen likely will not have a response to retreatment.

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Pentostatin or 2-deoxycoformycin (Nipent)

 

Approved by the FDA for hairy cell leukemia but is less active than 2-CDA for this disease. Treatment results in a 79% overall response rate and a 64% complete response rate in patients with hairy cell leukemia. Response rates (CR + PR) of more than 90% have been reported.

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Interferons

Class Summary

Interferons are naturally produced proteins with antitumor and immunomodulatory effects.

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Interferon alfa 2a (Roferon)

 

Protein product manufactured by recombinant DNA technology. First systemic drug shown to partially eradicate hairy cells from bone marrow, and first approved indication was for this disease. The mechanism of antitumor activity is not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of the host immune response may play important roles.

Roferon and Intron A differ from the natural product only in amino acid residue at position 23 and achieve similar results in hairy cell leukemia. Response rates are 65% overall, with 10% of patients achieving a complete remission.

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Interferon alfa-2b (Intron A)

 

Protein product manufactured by recombinant DNA technology. First systemic drug shown to partially eradicate hairy cells from bone marrow, and first approved indication was for this disease. The mechanism of antitumor activity is not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of the host immune response may play important roles.

Roferon and Intron A differ from the natural product only in amino acid residue at position 23 and achieve similar results in hairy cell leukemia. Response rates are 65% overall, with 10% of patients achieving a complete remission.

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Recombinant Granulocyte Colony-Stimulating Factors (G-CSFs)

Class Summary

Because treatment of hairy cell leukemia is associated with neutropenia, use of G-CSF may be helpful in reducing the toxicity of treatment for hairy cell leukemia.

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Filgrastim or G-CSF (Neupogen)

 

Shortens the early myelosuppressive effects of alfa interferon and reverses neutropenia in some patients with hairy cell leukemia.

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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.

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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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