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Artículo | IMSEAR | ID: sea-231046

RESUMEN

Introduction:T-PLL is a mature T-cell leukemia typically presenting at stages of exponentially rising lymphocyte counts in peripheral blood, accompanied by splenomegaly and bone marrow involvement.They are rare and inherently aggressive and notoriously refractory to therapeutics.To our knowledge, this is the largest series of T-PLLs from India. Objectives; We studied Immunophenot ypic characteristics, prognostic factors, outcomes, and treatments of 10 patients with T-PLL. Methods: Out of 4500 clinically suspected chronic leukemias, during 10 years, at Kidwai Memorial Institute of Oncology, which is a state cancer institute, diagnostic flow cytometric analysis was done and leukemias were classified based on WHO 2008 criteria, along with, morphology, cytogenetics, clinical, immunophenotyping and molecular findings. Results: out of 4500 cases of Chronic lymphoprolifer ative disorders sent for flow cytometric immunophenotyping, only 10 cases were diagnosed as T-PLL, accounting for 0.4 % mature leukemias of the lymphoid lineage. multiorgan involvement was common but effusion as a presenting feature was seen in only 10% of patients. Surprisingly skin involvement was evident in more number 70% of cases. single case showed cytogenetic abnormalities, later confirmed by FISH. Conclusions: Evaluation of the immunophenotype of this entity by flow cytometry is a critical part of diagnosis and is an indispensable tool in distinguishing T -PLL from other mature T -cell lymphoid neoplasms.

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