Skeletal Complications in Hairy Cell Leukemia Hairy cell leukemia is a rare B-cell lymphoproliferative disorder, accounting for 2 percent of the adult leukemias diagnosed annually in the United States. Clinical manifestations include pancytopenia, splenomegaly and the presence of typical hairy cells in the peripheral blood and bone marrow.
The clinical course of hairy cell leukemia is variable. Historically, infection, commonly caused by unusual organisms, has been the principal cause of morbidity and mortality in these patients. Skeletal involvement has been a rare complication, but may cause considerable symptomatic discomfort and pathologic fractures. Previously, splenectomy was the only treatment with proven efficacy for the palliation of hairy cell leukemia. In the past five years, however, interferon alfa and 2'-deoxycoformycin (pentostatin) have proved to be effective systematic therapies.
Lembersky and associates reviewed the records of 267 patients with hairy cell leukemia who were seen at the University of Chicago during a 12-year period and identified eight patients (3 percent) with skeletal complications. All of the patients were men; mean age of the group was 44 years. The median interval from diagnosis of hairy cell leukemia to the development of skeletal complications was 20 months. All of the patients complained of pain, and all but one lesion involved the axial skeleton, primarily the proximal femur. Lytic lesions were identified on radiographs in all but one patient. One patient also had multiple osteoporotic vertebral compression fractures. Radionuclide technetium bone scans were abnormal in all eight patients. Although the peripheral blood counts were variable (only two patients had a leukemic phase of the disease), all of the patients had hypercellular bone marrow biopsies with hairy cells that comprised at least 90 percent of the hematopoietic elements.
The skeletal abnormalities responded to local radiation therapy. Seven patients received systemic therapy with interferon alfa-2b following the diagnosis of the skeletal lesions. Four of the five evaluable patients demonstrated a partial hematologic response and substantial improvement in the degree of hairy cell infiltration of the bone marrow. None of these patients had recurrence of skeletal complications; median follow-up was 29 months. One patient failed to respond hematologically and developed additional bone lesions after one year of treatment. Another patient developed new skeletal lesions three months following the cessation of interferon therapy, at which time the bone marrow was packed with hairy cells.
The authors conclude that skeletal involvement is a rare complication of hairy cell leukemia and is associated with a high tumor burden in the bone marrow. In addition to local radiation therapy, systemic treatment with interferon alfa-2b should be considered. (Journal of Clinical Oncology, August 1988, vol. 6, p. 1280.)
COPYRIGHT 1989 American Academy of Family Physicians
COPYRIGHT 2004 Gale Group
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Hairy cell leukemia