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B-cell Prolymphocytic Leukemia: Case Report and Challenges on a Diagnostic and Therapeutic Forefront
B-cell prolymphocytic leukemia (B-PLL) is a rare malignancy of mature B-cells with characteristic morphologic, immunophenotypic, cytogenetic, and molecular features characterized by late onset (median age 69 years), an aggressive clinical course, refractoriness to chemotherapy, and median survival o...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6822919/ https://www.ncbi.nlm.nih.gov/pubmed/31700732 http://dx.doi.org/10.7759/cureus.5629 |
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author | Bindra, Bikramjit S Kaur, Harpreet Portillo, Shellsea Emiloju, Oluwadunni Garcia de de Jesus, Katherine |
author_facet | Bindra, Bikramjit S Kaur, Harpreet Portillo, Shellsea Emiloju, Oluwadunni Garcia de de Jesus, Katherine |
author_sort | Bindra, Bikramjit S |
collection | PubMed |
description | B-cell prolymphocytic leukemia (B-PLL) is a rare malignancy of mature B-cells with characteristic morphologic, immunophenotypic, cytogenetic, and molecular features characterized by late onset (median age 69 years), an aggressive clinical course, refractoriness to chemotherapy, and median survival of around three years. Treatment is influenced by the presence or absence of specific high-risk genetic mutations like 17P/TP53 deletion, the presence of which translates into poor prognosis. Patients without 17P deletion, who are <70 years, without significant co-morbidities, are initially treated with a combination chemotherapy regimen used for chronic lymphocytic leukemia (CLL) such as fludarabine, cyclophosphamide, and rituximab. On the other hand, patients with a 17P deletion, age >70 years, with multiple co-morbidities, receive ibrutinib or alemtuzumab as the initial therapy. Relapsed or refractory cases are managed with BCL-2 signaling inhibitors like venetoclax. We discuss the case of an 84-year-old male with B-PLL (positive TP53 mutation), resistant to ibrutinib therapy, with extremely high white blood cell (WBC) counts, thus creating a dilemma regarding the best treatment in the second-line setting. |
format | Online Article Text |
id | pubmed-6822919 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-68229192019-11-07 B-cell Prolymphocytic Leukemia: Case Report and Challenges on a Diagnostic and Therapeutic Forefront Bindra, Bikramjit S Kaur, Harpreet Portillo, Shellsea Emiloju, Oluwadunni Garcia de de Jesus, Katherine Cureus Oncology B-cell prolymphocytic leukemia (B-PLL) is a rare malignancy of mature B-cells with characteristic morphologic, immunophenotypic, cytogenetic, and molecular features characterized by late onset (median age 69 years), an aggressive clinical course, refractoriness to chemotherapy, and median survival of around three years. Treatment is influenced by the presence or absence of specific high-risk genetic mutations like 17P/TP53 deletion, the presence of which translates into poor prognosis. Patients without 17P deletion, who are <70 years, without significant co-morbidities, are initially treated with a combination chemotherapy regimen used for chronic lymphocytic leukemia (CLL) such as fludarabine, cyclophosphamide, and rituximab. On the other hand, patients with a 17P deletion, age >70 years, with multiple co-morbidities, receive ibrutinib or alemtuzumab as the initial therapy. Relapsed or refractory cases are managed with BCL-2 signaling inhibitors like venetoclax. We discuss the case of an 84-year-old male with B-PLL (positive TP53 mutation), resistant to ibrutinib therapy, with extremely high white blood cell (WBC) counts, thus creating a dilemma regarding the best treatment in the second-line setting. Cureus 2019-09-11 /pmc/articles/PMC6822919/ /pubmed/31700732 http://dx.doi.org/10.7759/cureus.5629 Text en Copyright © 2019, Bindra et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Oncology Bindra, Bikramjit S Kaur, Harpreet Portillo, Shellsea Emiloju, Oluwadunni Garcia de de Jesus, Katherine B-cell Prolymphocytic Leukemia: Case Report and Challenges on a Diagnostic and Therapeutic Forefront |
title | B-cell Prolymphocytic Leukemia: Case Report and Challenges on a Diagnostic and Therapeutic Forefront |
title_full | B-cell Prolymphocytic Leukemia: Case Report and Challenges on a Diagnostic and Therapeutic Forefront |
title_fullStr | B-cell Prolymphocytic Leukemia: Case Report and Challenges on a Diagnostic and Therapeutic Forefront |
title_full_unstemmed | B-cell Prolymphocytic Leukemia: Case Report and Challenges on a Diagnostic and Therapeutic Forefront |
title_short | B-cell Prolymphocytic Leukemia: Case Report and Challenges on a Diagnostic and Therapeutic Forefront |
title_sort | b-cell prolymphocytic leukemia: case report and challenges on a diagnostic and therapeutic forefront |
topic | Oncology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6822919/ https://www.ncbi.nlm.nih.gov/pubmed/31700732 http://dx.doi.org/10.7759/cureus.5629 |
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