The clinico-hematological profile of hairy cell leukaemia: a single centre experience.
ABSTRACT The response rates and overall survival of hairy cell leukemia has changed remarkably with cladribine. Twenty patients diagnosed as hairy cell leukemia over a 5 year period were evaluated. Median age of the patients was 52.5 years. Splenomegaly was seen in 85% and hepatomegaly in 50% patients. At presentation, pancytopenia was seen in 45% and bicytopenia in 20% patients. Tartrate resistant acid phosphatase was positive in 73.68%. Cladribine was given in 13 patients. Overall survival was 84.6%. Median duration of follow-up was 17 months (4-108 months). Direct Coombs' test positive autoimmune hemolytic anemia was seen in two patients (15.38%) on cladribine therapy who required treatment with steroids.
- [show abstract] [hide abstract]
ABSTRACT: Hairy cell leukemia (HCL) is an uncommon, indolent, chronic B-cell lymphoproliferative disorder involving the marrow and spleen. Therapy for HCL includes splenectomy, interferon alfa-2a and alfa-2b, pentostatin, and cladribine. The purpose of this article was to report the extended follow-up of HCL patients treated with cladribine. Two hundred nine patients with HCL who were treated with cladribine had at least 7 years of follow-up. A course of cladribine constituted a 7-day continuous intravenous infusion at a dose of 0.1 mg/kg/d. Of the 207 assessable patients who had at least 7 years of follow-up, 196 (95%) achieved a complete response (CR) and 11 (5%) achieved a partial response (PR) after a single course of cladribine (overall response rate, 100%). The median first-response duration for all responders was 98 months. Seventy-six patients (37%) experienced relapse after their first course of cladribine. The median time to first relapse for all responders was 42 months. Time to treatment failure of CRs compared with PRs was statistically significant (P <.0005). The overall survival rate was 97% recorded at 108 months. Forty-seven patients developed 58 second malignancies. The observed-to-expected ratio for second malignancies was 2.03 (95% confidence interval, 1.49 to 2.71). These results confirm previous observations that single courses of cladribine administered to patients with HCL induce high response rates, the majority of which are CRs. Most patients enjoy long-lasting complete remissions, and those patients who experience relapse can be successfully re-treated with cladribine.Journal of Clinical Oncology 03/2003; 21(5):891-6. · 18.04 Impact Factor
- [show abstract] [hide abstract]
ABSTRACT: Hairy cell leukemia is a rare disease, probably of B-lymphocyte origin, that has been reported to rarely occur with polyarteritis nodosa. The records of 31 patients with hairy cell leukemia were reviewed for an association with other disorders of the immune system; such an association was found more often than previously suspected. Four cases are discussed--one with hepatitis B surface antigen-positive polyarteritis nodosa and another with an IgA (kappa) monoclonal gammopathy and amyloidosis of the kidney, liver, and small bowel. The other two patients had cutaneous vasculitis, one of which was a rare form of leukocytoclastic angiitis--erythema elevatum diutinum. Because the number of patients in this study is small, it is impossible to say whether the association of hairy cell leukemia with any of these immunologic disorders adversely affects survival.Archives of Internal Medicine 06/1982; 142(5):902-3. · 11.46 Impact Factor
Article: The biology of hairy cells.[show abstract] [hide abstract]
ABSTRACT: The evidence that hairy cells are activated clonal late B cells is presented. The largely non-specific (i.e. not confined to hairy-cell leukaemia) chromosome and genetic abnormalities are then described. Next, the features of malignant-cell activation are considered, including the distinctive morphology of hairy cells and their expression of activation-related antigens and activated adhesion receptors. Also, signalling and cytokine production are discussed in the context of malignant-cell activation. It is then demonstrated that many of the distinctive clinicopathological features of hairy-cell leukaemia can be explained in terms of the interaction of the activated malignant cells with other types of cell and tissue matrix. Finally, the biological basis of the hairy cell's unusually high sensitivity to IFN-alpha and nucleoside analogues is discussed.Bailliè re s Best Practice and Research in Clinical Haematology 04/2003; 16(1):1-13. · 2.81 Impact Factor