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Originally published as JCO Early Release 10.1200/JCO.2008.20.3372 on December 1 2008 © 2009 American Society of Clinical Oncology.
Teenager With Hairy Cell Leukemia: 30-Year Follow-Up
Permanente Medical Center, South San Francisco, CA
Department of Dermatology, Roger Williams Medical Center, Providence, RI To the Editor: We read with interest the recent commentary on hairy cell leukemia (HCL) by Golomb,1 published 25 years after one of his previous articles on the disease, in which he stated that "the disease occurs in patients from 20 to over 80 years of age, but has not been reported in a child or teenager."2 We have observed a patient diagnosed at age 18 years with HCL for more than 30 years, and wish to summarize his case briefly. An 18-year-old white man was first evaluated in March 1977 for gum bleeding, abdominal discomfort, and pancytopenia. Physical examination revealed pallor, scattered ecchymoses, and splenomegaly. Laboratory studies revealed a hematocrit of 30.6%; platelet count of 30,600/µL; and leukocyte count of 1,900/µL, with 44% granulocytes and 54% lymphocytes, a small number of which had hairy projections consistent with HCL. A bone marrow biopsy confirmed the diagnosis of HCL. A splenectomy was performed in April 1977, with removal of a 1,765-g spleen, which had features typical of HCL on histologic examination (Fig 1).
After splenectomy, the patient's blood counts returned to normal, and he felt well for 8 years until November 1985, when he noted fatigue. After a syncopal episode 1 month later, he was hospitalized, and the blood counts showed a hematocrit of 10.5%; platelet count of 22,000/µL; and leukocyte count of 3,600/µL, with 45% granulocytes and 37% lymphocytes, 20% of which had features of hairy cells. Bone marrow biopsy was 75% cellular, with diffuse infiltration by a monotonous population of small mononuclear cells consistent with HCL. It was estimated that 90% of the marrow cellularity was HCL. Horning of Stanford Medical Center (Stanford, CA) initiated an interferon protocol in February 1986, and treatment with human lymphoblastoid interferon was initiated at a dose of 2 MU/m2 three times a week for 6 months. The patient was secondarily randomly selected to receive a second 6 months of treatment, after which he was deemed by bone marrow biopsy to be in complete remission, with normal blood counts (granulocytes, 9.1/µL; hemoglobin, 15.1 g/dL; platelet count, 448,000/µL). The patient is now working full time and continues to be asymptomatic, but has mild liver function abnormalities and serologic evidence of hepatitis C, possibly related to platelet transfusions given at the time of splenectomy. His blood counts remain normal, and a peripheral-blood analysis in June 2006 showed no evidence of an abnormal lymphocyte clone. Because of the patient's young age, his initial pathology specimens were reviewed by pathologists at University of California, San Francisco (M.E.K.; San Francisco, CA); Stanford University (Stanford, CA); University of Chicago (Chicago, IL); and Harvard Medical School (Boston, MA), and were believed to be typical of HCL. To our knowledge, this patient is the youngest presenting with HCL yet recorded.3-5 Although purine analogs are now the usual first-line treatment for HCL, this patient's clinical course demonstrates again the possibility of prolonged remissions after splenectomy and interferon treatment. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
NOTES published online ahead of print at www.jco.org on December 1, 2008 REFERENCES
1. Golomb HM: Hairy cell leukemia: Treatment successes in the last 25 years. J Clin Oncol 26:2607-2609, 2008 2. Golomb HM: Hairy cell leukemia: Lessons learned in twenty-five years. J Clin Oncol 1:652-656, 1983[Abstract] 3. O’Brien SO, Keating MJ: Chronic leukemias, in DeVita BR, Hollman S, Rosenberg SA (eds): Cancer: Principles and Practice of Oncology (ed 7). Philadelphia, PA, Lippincott Williams & Wilkins, 2005, pp 2133-2144 4. Johnston JB: Hairy cell leukemia, in Lee GR, Foerster J, Lukens J, et al (eds): Wintrobe's Clinical Hematology (ed 10). Baltimore, MD, Lippincott Williams & Wilkins, 1999, pp 2428-2446 5. Saven A: Hairy cell leukemia, in Lichtman MA, Beutler E, Kaushansky K, et al (eds): Williams Hematology (ed 7). New York, NY, McGraw-Hill, 2005, pp 1385-1393
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Copyright © 2009 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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