Dr Namrata TRUE TALES OF TEN T-PLL OVER A DECADE
T- prolymphocytic leukemia(T-PLL) ,are aggressive lymphoid malignancies with unique morphologic, cytogenetic, immunophenotypic, and molecular features. 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 extremely rare ,comprising of <2% of mature T –leukemias and inherently aggressive and notoriously refractory to therapeutics.Extensive search for Indian literature on T-PLL and Immunophenotypic characterization of this entity did not yield any results. We analysed the characteristics, prognostic factors, outcomes and treatments of 10 patients with T-PLL . To our knowledge this is the largest series of T-PLL from India , this study reviews clinical, cytogentic and flow cytometric immunophenotyping findings, and differential diagnosis of patients with T-cell prolymphocytic leukemia from January 2012 to January 2022.Methods: Our laboratory received 4500 clinically suspected chronic leukemias, during a period of 10 years ,at kidwai memorial institute of oncology ,which is state cancer institute , where diagnostic flow cytometric analysis was done and leukemias were classified based on WHO 2008 criterias. Results: out of 4500 cases of Chronic lympho proliferative disorders sent for flow cytometric immunophenotyping, only 10 cases were diagnosed as T-PLL .Study period January 2012 to January 2022.Conclusions: Evaluation of immunophenotype of this rare entity by flow cytometry is a critical part of diagnosis and is a indespensible tool in distinguishing T-PLL from other mature T-cell lymphoid neoplasms.Awareness of this rare entity aids in work up .
• 2. Matutes E, Catovsky D, Mature T-cell leukemias and leukemia/ lymphoma syndromes. Review of our experience in 175 cases. Leuk Lymphoma 1991 ;volume 4: issue 2,81-91 https://doi.org/10.3109/10428199109068049
• 3. .P.Jain, E.Aoki , M.Keating et al. Characteristics, outcomes , prognostic factors and treatment of patients with T-cell pro lymphocytic leukemia (T -PLL). Annals of Oncology2017;Volume 28: 1554 – 1559. 10.1093/annonc/mdx163
• 4.Marco Herling, Joseph D Khoury, LaBaron T et al. A systematic approach to diagnosis of mature T-cell leukemias reveals heterogeneity among WHO categories. Blood 2004;volume 104:number 2:328-335 10.1182/blood-2004-01-0002
5.Xueyan Chen , Sindhu Cherian . Immuno phenotypic Characterization of T-Cell Pro lymphocytic Leukemia. Am J Clin Pathol 2013;140: 727-735 https://doi.org/10.1309/AJCPG71KYOXTKLQW
6.Pawson R, Matutes E , Brito-Babapulle V et al. Sezary cell leukaemia: a distinct T cell disorder or a variant form of T pro-lymphocytic leukaemia?.Leukemia 1997;11: 1009-1013 https://doi.org/10.1038/sj.leu.2400710
7.Matutes E, Brito-Babapulle V,Swansbury J et al.Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia. Blood 1991;volume78: issue 12:3269-3274 https://doi.org/10.1182/blood.V78.12.3269.3269
8. Bain BJ, Barnett D, Linch D, Matutes E, Reily JT. Revised guideline on immuno phenotyping in acute leukemia and chronic lympho proliferative disorders.Clin.Lab.Haem2002;24:1-13 https://doi.org/10.1046/j.1365-2257.2002.00135.x
9.Shano Naseem. Ritu gupta,Rajesh kashyap et al .T-Cell pro lymphocytic leukemia;a report of 2 cases with review of literature .indian journal of hematology and blood transfusion 2008;24:178-181
10. Herling M, Patel KA, Teitell MA, et al. High TCL1 expression and intact T-cell receptor signaling define a hyperproliferative subset of T-cell pro loymphocytic leukemia. Blood 2008; 111(1): 328-337 https://doi.org/10.1182/blood-2007-07-101519
11.Philipp B Staber, Marco Herling, Mar Bellido. Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell pro lymphocytic leukemia. Blood. 2019 Oct 3; 134(14): 1132–1143. doi: 10.1182/blood.2019000402.
12. Dearden CE,Khot A, Else M, et al. Alemtuzumab therapy in T-cell prolymphocytic leukemia:comparing efficacy in a series treated intravenously and a study piloting the subcutaneous route. Blood 2011; 118(22):5799-5802. https://doi.org/10.1182/blood-2011-08-372854
13. Till Braun , Jana von Jan, Linus Wahnschaffe , et al. Advances and Perspectives in the treatment of T-PLL. Current Hematologic Malignancy Reports 2020:15(2):113-124. doi: 10.1007/s11899-020-00566-5
14. Alexandra Schrader , Till Braun and Marco Herling . The dawn of a new era in treating T-PLL . Oncotarget,2019;10: 626-628. https://doi.org/10.18632/oncotarget.26595
Copyright (c) 2023 Author (s). Published by Siddharth Health Research and Social Welfare Society
This work is licensed under a Creative Commons Attribution 4.0 International License.