T-Cell Prolymphocytic Leukemia: An Experience From A Tertiary Cancer Centre In South India.

Background - T-cell prolymphocytic leukemia (T-PLL) is a rare lymphoid malignancy with dismal prognosis. Most patients have increased lymphocyte count (>1, 00, 000/dL) and widespread disease at presentation. Despite high response rate seen with alemtuzumab, the disease relapse is inevitable. Methods - This was a retrospective observational study done at a tertiary cancer centre in South India. All patients diagnosed with T-PLL from August 2010 to July 2015 were studied for the clinical characteristics, pathological findings and treatment outcomes. Results - Seven patients were diagnosed as T-PLL over a period of 5 years. The median age at diagnosis was 51 years. In the present series, 6 patients (86%) had splenomegaly and 3 had hepatomegaly (43%). Generalized lymphadenopathy was seen in 4 (57%) patients at presentation. Skin lesions were seen in 5 (71%) patients whereas pleural effusion in only one patient (14%). All had elevated total leukocyte count, with more than 1, 00, 000/ dL in 4 patients. The median survival was 5 months with different chemotherapy (CT) regimens (5 received CT and 2 patients on best supportive care). Conclusion - T-PLL is a rare diagnosis with no definite treatment guidelines. Treatment with alemtuzumab and stem cell transplant have the best outcome at present, but invariably the disease relapse. [ABSTRACT FROM AUTHOR]