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Leukemia & Lymphoma, June 2011; 52(S2): 18–20


Historical treatments of in hairy cell leukemia, splenectomy and interferon: past and current uses


THOMAS M. HABERMANN1 & KANTI RAI2


1Division of Hematology, Department of Medicine, Mayo Clinic College of Medicine and Mayo Foundation, Rochester, MN, USA and 2Division of Hematology, Long Island Jewish Medical Center and Albert Einstein College of Medicine, New York, NY, USA


Abstract The management of hairy cell leukemia has undergone historic unprecedented changes since three decades ago. This review summarizes treatment approaches utilized before the adenosine deaminase inhibitors. These historic approaches may still be utilized in patients with relapsed or refractory disease.


Keywords: Hairy cell leukemia, splenectomy in hairy cell leukemia Introduction


The treatment of hairy cell leukemia has evolved over time. Observation remains an indication in some patients. Previous modalities include corticosteroids, chlorambucil, methotrexate, lithium carbonate, an- drogens, leukapheresis, combination chemotherapy, radiation therapy, and bone marrow transplant. Present modalities include splenectomy, interferon, pentostatin, 2-chlorodeoxyadenosine, rituximab, and anti-CD22 with recombinant immunotoxin. Readers are referred to a more extensive review [1].


Treatments of historical interest


Leukapheresis, introduced in 1965, results in a transient decrease in the white blood cell count with no impact on the bone marrow. Lithium carbonate produced a mild benefit for leukopenia in some patients. Androgens (halotestin, oxymetho- lone) were utilized for anemia. Single-agent alkylator chemotherapy with chlorambucil was incorporated with minimal improvement in peripheral blood counts in the majority of patients treated. Combina- tion chemotherapy with regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, and


prednisone) produced severe cytopenias and did not reverse the bone marrow infiltrate. Allogeneic marrow transplant with identical twins


was reported and was successful. The utilization of corticosteroids and systemic vasculitis in hairy cell leukemia was effective. Radiation therapy has been demonstrated to be active for lytic bone lesions. High-dose methotrexate was reported to be effective in patients with an elevated white blood cell count.


Single-agent alkylator chemotherapy


Single-agent alkylator chemotherapy with chloram- bucil was most efficacious in leukemic relapses, but neutropenia and leukopenia did not improve [2]. The bone marrow did not normalize. Myelodysplas- tic syndromes were reported [3]. Survival after observation, splenectomy, and chlorambucil was 68% at 5 years in 65 patients [4].


Observation


In 2010, there are no standard guidelines for observation. This is now an especially important issue at the time of relapse. Decisions are based upon minimal disease, age, associated comorbidities,


Correspondence: Thomas M. Habermann, MD, Mayo Clinic, W10A, 200 First Street SW, Rochester, MN 55905, USA. Tel: 507-266-2041. Fax: 507-266-4972. E-mail: Habermann.thomas@mayo.edu


ISSN 1042-8194 print/ISSN 1029-2403 online  2011 Informa UK, Ltd. DOI: 10.3109/10428194.2011.573033


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