eMedicine Specialties > Hematology > Stem Cells and Disorders

Hairy Cell Leukemia: Follow-up

Author: Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Coauthor(s): Ulrich Woermann, MD, Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland
Contributor Information and Disclosures

Updated: Nov 24, 2008

Follow-up

Further Inpatient Care

  • Hyperuricemia may occur during therapy in patients with hairy cell leukemia with leukocytosis and high tumor burden. Add allopurinol at 300 mg per day orally.
  • The risk of second malignancies has been observed in affected patients either through hairy cell leukemia disease itself or secondary from the immunosuppressive effects of the therapy, including melanoma, prostate cancers, gastrointestinal malignancies, non-Hodgkin lymphomas, and nonmelanomatous cancers.
  • A 20-year follow-up in 117 patients in British Columbia showed 31% developed a second malignancy, of which 30% were diagnosed before hairy cell leukemia was found.11 On the other hand, MD Anderson reported no excess of second malignancies among 350 patients with hairy cell leukemia who were treated with interferon, 2'-CdA, or 2'-DCF.12

Further Outpatient Care

  • Hairy cell leukemia is usually indolent and protracted; late relapses occur.
  • Long-term outpatient follow-up is often necessary in most patients.
  • The overall survival rate with 2'DCF up front or after alpha interferon failure in 241 patients was 80-85% at 10 years.
  • Evaluation of minimal residual disease by posttreatment bone marrow biopsies using anti-CD20 by flow cytometry reveals that 13-51% of patients in apparent CR had minimal residual disease and appears to predict clinical relapse. Because a majority respond very well to retreatment (92% response) or salvage treatment (80% response), no evidence supports treatment of minimal residual disease.
  • Newer therapies, such as anti-CD20 monoclonal antibody rituximab, had been tested in patients with hairy cell leukemia that was refractory to standard treatment. Several studies with small numbers of patients who received rituximab showed an overall response of 64%, with a median duration of response of 14 months, to 100% response and a duration of 73 months, indicating that this form of therapy is active against hairy cell leukemia.

Prognosis

  • Hairy cell leukemia behaves like a chronic leukemia. With new therapies, most patients achieve clinical remissions and, sometimes, long-term cures.
  • Although relapses are known to occur after 5-10 years, they are usually responsive to the same treatment.
  • Most of the difficulty is in making the diagnosis.

Patient Education

 


More on Hairy Cell Leukemia

Overview: Hairy Cell Leukemia
Differential Diagnoses & Workup: Hairy Cell Leukemia
Treatment & Medication: Hairy Cell Leukemia
Follow-up: Hairy Cell Leukemia
Multimedia: Hairy Cell Leukemia
References
Further Reading
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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. 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].

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

  5. 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].

  6. 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].

  7. 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].

  8. 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].

  9. 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].

  10. 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].

  11. 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].

  12. 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].

  13. 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].

  14. 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].

  15. 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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Keywords

hairy cell leukemia, hairy cell, leukemic reticuloendotheliosis, HCL, chronic lymphoid leukemia, lymphoproliferative disorders, B-cell disease, clonal B-cell lymphocyte, pancytopenia, anemia, thrombocytopenia, neutropenia, hepatosplenomegaly

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Contributor Information and Disclosures

Author

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, 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 Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Coauthor(s)

Ulrich Woermann, MD, Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland
Disclosure: Nothing to disclose.

Medical Editor

Rodger L Bick, MD, PhD, FACP, Clinical Professor of Medicine, University of Texas Southwestern Medical Center; 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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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.

CME Editor

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, Program Director, Hematology-Oncology Fellowship, 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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