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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| Format: | Article |
| Language: | English |
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Tehran University of Medical Sciences
2018-04-01
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| Series: | International Journal of Hematology-Oncology and Stem Cell Research |
| Subjects: | |
| Online Access: | https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/814 |
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| author | Suresh Babu MC Abhishek Anand Kuntegowdanahalli C. Lakshmaiah Govind Babu K Dasappa Lokanatha Linu Abraham Jacob DS Madhumathi Kadabur N Lokesh AH Rudresha LK Rajeev Rajesh Patidar |
| author_facet | Suresh Babu MC Abhishek Anand Kuntegowdanahalli C. Lakshmaiah Govind Babu K Dasappa Lokanatha Linu Abraham Jacob DS Madhumathi Kadabur N Lokesh AH Rudresha LK Rajeev Rajesh Patidar |
| author_sort | Suresh Babu MC |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-12T10:45:28Z |
| format | Article |
| id | doaj.art-4546dfbdd74b4703b21b68add4a717e5 |
| institution | Directory Open Access Journal |
| issn | 2008-2207 |
| language | English |
| last_indexed | 2024-03-12T10:45:28Z |
| publishDate | 2018-04-01 |
| publisher | Tehran University of Medical Sciences |
| record_format | Article |
| series | International Journal of Hematology-Oncology and Stem Cell Research |
| spelling | doaj.art-4546dfbdd74b4703b21b68add4a717e52023-09-02T07:32:39ZengTehran University of Medical SciencesInternational Journal of Hematology-Oncology and Stem Cell Research2008-22072018-04-01122T-Cell Prolymphocytic Leukemia: An Experience from a Tertiary Cancer Centre in South IndiaSuresh Babu MC0Abhishek Anand1Kuntegowdanahalli C. Lakshmaiah2Govind Babu K3Dasappa Lokanatha4Linu Abraham Jacob5DS Madhumathi6Kadabur N Lokesh7AH Rudresha8LK Rajeev9Rajesh Patidar10Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaDepartment of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaDepartment of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaDepartment of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaDepartment of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaDepartment of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaDepartment of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaDepartment of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaDepartment of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaDepartment of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaDepartment of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, IndiaBackground: 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.https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/814T-cellsProlymphocytic leukemiaAlemtuzumab |
| spellingShingle | Suresh Babu MC Abhishek Anand Kuntegowdanahalli C. Lakshmaiah Govind Babu K Dasappa Lokanatha Linu Abraham Jacob DS Madhumathi Kadabur N Lokesh AH Rudresha LK Rajeev Rajesh Patidar T-Cell Prolymphocytic Leukemia: An Experience from a Tertiary Cancer Centre in South India International Journal of Hematology-Oncology and Stem Cell Research T-cells Prolymphocytic leukemia Alemtuzumab |
| 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 | T-cells Prolymphocytic leukemia Alemtuzumab |
| url | https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/814 |
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