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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. Materia...

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Autores principales: Babu MC, Suresh, Anand, Abhishek, Lakshmaiah, Kuntegowdanahalli C., Babu K, Govind, Lokanatha, Dasappa, Jacob, Linu Abraham, Madhumathi, DS, Lokesh, Kadabur N, Rudresha, AH, Rajeev, LK, Patidar, Rajesh
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Tehran University of Medical Sciences, Hematology-Oncology and Stem Cell Transplantation Research Center 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6141430/
https://www.ncbi.nlm.nih.gov/pubmed/30233775
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author Babu MC, Suresh
Anand, Abhishek
Lakshmaiah, Kuntegowdanahalli C.
Babu K, Govind
Lokanatha, Dasappa
Jacob, Linu Abraham
Madhumathi, DS
Lokesh, Kadabur N
Rudresha, AH
Rajeev, LK
Patidar, Rajesh
author_facet Babu MC, Suresh
Anand, Abhishek
Lakshmaiah, Kuntegowdanahalli C.
Babu K, Govind
Lokanatha, Dasappa
Jacob, Linu Abraham
Madhumathi, DS
Lokesh, Kadabur N
Rudresha, AH
Rajeev, LK
Patidar, Rajesh
author_sort Babu MC, Suresh
collection PubMed
description 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. Materials and Methods : This was a retrospective observational study done at a tertiary cancer center 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 was seen 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 patients treated with CT and 2 received best supportive care). Conclusion: T-PLL is a rare disease with no definite treatment guidelines. At present, the best outcomes are achieved if treatment with alemtuzumab is followed by stem cell transplant, but the disease invariably relapses. Countries where affordability remains a big challenge, the best approach needs to be defined beyond the monoclonal antibodies and transplant.
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spelling pubmed-61414302018-09-19 T-Cell Prolymphocytic Leukemia: An Experience from a Tertiary Cancer Centre in South India Babu MC, Suresh Anand, Abhishek Lakshmaiah, Kuntegowdanahalli C. Babu K, Govind Lokanatha, Dasappa Jacob, Linu Abraham Madhumathi, DS Lokesh, Kadabur N Rudresha, AH Rajeev, LK Patidar, Rajesh Int J Hematol Oncol Stem Cell Res Original Article 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. Materials and Methods : This was a retrospective observational study done at a tertiary cancer center 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 was seen 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 patients treated with CT and 2 received best supportive care). Conclusion: T-PLL is a rare disease with no definite treatment guidelines. At present, the best outcomes are achieved if treatment with alemtuzumab is followed by stem cell transplant, but the disease invariably relapses. Countries where affordability remains a big challenge, the best approach needs to be defined beyond the monoclonal antibodies and transplant. Tehran University of Medical Sciences, Hematology-Oncology and Stem Cell Transplantation Research Center 2018-04-01 /pmc/articles/PMC6141430/ /pubmed/30233775 Text en Copyright : © International Journal of Hematology-Oncology and Stem Cell Research & Tehran University of Medical Sciences This is an Open Access article distributed under the terms of the Creative Commons Attribution License, (http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Article
Babu MC, Suresh
Anand, Abhishek
Lakshmaiah, Kuntegowdanahalli C.
Babu K, Govind
Lokanatha, Dasappa
Jacob, Linu Abraham
Madhumathi, DS
Lokesh, Kadabur N
Rudresha, AH
Rajeev, LK
Patidar, Rajesh
T-Cell Prolymphocytic Leukemia: An Experience from a Tertiary Cancer Centre in South India
title T-Cell Prolymphocytic Leukemia: An Experience from a Tertiary Cancer Centre in South India
title_full T-Cell Prolymphocytic Leukemia: An Experience from a Tertiary Cancer Centre in South India
title_fullStr T-Cell Prolymphocytic Leukemia: An Experience from a Tertiary Cancer Centre in South India
title_full_unstemmed T-Cell Prolymphocytic Leukemia: An Experience from a Tertiary Cancer Centre in South India
title_short T-Cell Prolymphocytic Leukemia: An Experience from a Tertiary Cancer Centre in South India
title_sort t-cell prolymphocytic leukemia: an experience from a tertiary cancer centre in south india
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6141430/
https://www.ncbi.nlm.nih.gov/pubmed/30233775
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