Leukemia & Lymphoma, June 2011; 52(S2): 3–5
Fifty years of hairy cell leukemia treatments
HARVEY M. GOLOMB Section of Hematology/Oncology, University of Chicago, Chicago, IL, USA
Abstract Treatment of hairy cell leukemia (HCL), a disease first described in 1958, has evolved from splenectomy, which resulted in a normalization of blood counts in about 41% of patients and an improvement in the remaining 59% of patients but with a time to failure of only approximately 19 months, through treatment in the early 1980s with interferon, which resulted in the same high overall response rate but with a time to failure of approximately 31 months. Subsequently, therapy with either pentostatin or cladribine showed an increase in the complete remission (CR) rate to approximately 80–90%, with only a small percentage of patients relapsing at approximately 30 months. More recently, patients who have failed either or both of these drugs have been shown to respond to rituximab or the experimental drug, BL22 (HA22). With these documented successes, the outlook for patients diagnosed with HCL, 50 years after the disease was first described, is so positive that patients with HCL have survival curves similar to those for the appropriate age-related cohorts.
Keywords: hairy cell leukemia, pentostatin, cladribine, rituximab
In 1958, Bouroncle and colleagues at Ohio State University first described hairy cell leukemia, a malignant lymphoproliferative disease of B-cell origin, as leukemic reticuloendotheliosis, a distinct clinical–pathologic entity. By the mid-1970s it had become a more easily recognized disease, character- ized by splenomegaly and pancytopenia without lymphadenopathy. Peripheral blood smears showed a low percentage of abnormal mononuclear cells with irregular cytoplasmic projections. The cells were present in many organs, but were of pathophysiologic significance in their presence in the bone marrow and spleen. The disease constitutes approximately 2% of the adult leukemias and occurs more often in men than women (ratio of 4:1). The best approach to diagnosis is to examine blood and bone marrow biopsy specimens to identify cells with the morpho- logic features of hairy cells and to demonstrate that the neoplastic cells have an antigenic profile that is characteristic for HCL. With transmission electron microscopy, a ribosome–lamella complex can be found in the cytoplasm of approximately 50% of cases. The clinical features of hairy cell leukemia
(HCL) were reviewed in an early series of 71 patients from three institutions in 1978 [1]. Of the 59 patients without previous splenomegaly,
35(59%) had pancytopenia, 10 (17%) were in the leukemic phase of the disease, and 14 (24%) had a decrease in one or two of the blood counts. A palpable spleen was present in 83% of patients, but peripheral adenopathy was insignificant. Cytopenias result from a combination of splenic sequestration and bone marrow underproduction owing to leukemic infiltration. The initial treatment consid- ered to reverse the cytopenias consisted of splenectomy. Splenectomy was attractive because it addressed the problem of splenic sequestration. A report on the response to splenectomy in 65 patients with HCL at one institution was published in 1983 [2]. Thirty-seven patients (41%) had a complete remission (CR) as defined by a return in white blood cell (WBC), red blood cell (RBC), and platelet counts to a defined level, and 38 (59%) had a partial remission (PR) with a return of only one or two of those parameters to a defined level. The 5-year actuarial survival for all patients was 68%, for patients with CR it was 76%, and for patients with PR 62%. It was shown that spleen size did not affect the response to splenectomy. Extensive bone marrow involvement was more predictive of an inadequate hematologic response to splenectomy. Overall, approximately 65% of patients eventually progressed after splenectomy.
Correspondence: Harvey M. Golomb, MD, Section of Hematology/Oncology, University of Chicago, Chicago, IL 60637, USA. E-mail:
hgolomb@medicine.bsd.uchicago.edu
ISSN 1042-8194 print/ISSN 1029-2403 online 2011 Informa UK, Ltd. DOI: 10.3109/10428194.2011.565094
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