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CB-LPD, MGUS, T-LGLL, and PRCA: A rare case report of 4 concomitant hematological disorders
RATIONALE: Monoclonal gammopathy of undetermined significance (MGUS) is a clinically asymptomatic clonal plasma cell or lymphoplasmacytic proliferative disorder. Recently, some case reports have described the association of pure red cell aplasia (PRCA) with MGUS, even with a relatively low monoclona...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Lippincott Williams & Wilkins
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8615434/ https://www.ncbi.nlm.nih.gov/pubmed/34964755 http://dx.doi.org/10.1097/MD.0000000000027874 |
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author | Xu, Qinhong Yu, Jieni Lin, Xiaoyan Li, Youli Zhang, Kejie |
author_facet | Xu, Qinhong Yu, Jieni Lin, Xiaoyan Li, Youli Zhang, Kejie |
author_sort | Xu, Qinhong |
collection | PubMed |
description | RATIONALE: Monoclonal gammopathy of undetermined significance (MGUS) is a clinically asymptomatic clonal plasma cell or lymphoplasmacytic proliferative disorder. Recently, some case reports have described the association of pure red cell aplasia (PRCA) with MGUS, even with a relatively low monoclonal immunoglobulin burden. T large granular lymphocyte leukemia (T-LGLL) is a chronic lymphoproliferative disorder characterized by clonal expansion of T large granular lymphocytes, which is rare in China. There are some reports about T-LGL leukemia in patients with B-cell lymphoma; however, it is very rare that T-LGLL coexists with MGUS and clonal B-cell lymphoproliferative disorders (CB-LPD). PATIENT CONCERNS: A 77-year-old man was hospitalized because of anemia. He was diagnosed with MGUS, CB-LPD, and PRCA. During the development of the disease, a group of abnormal T lymphocytes was detected by flow cytometry of peripheral blood. DIAGNOSIS: Combining clinical manifestations with the result of T cell receptor gene rearrangement and immunophenotype, it was consistent with the diagnosis of T large granular lymphocyte leukemia. INTERVENTIONS: The patient was treat with bortezomib and dexamethasone regimen, Rituximab and sirolimus. OUTCOMES: The patient was transfusion independent after therapies. LESSONS: We report a patient with 4 concomitant hematological disorders: T-LGLL, MGUS, CB-LPD, and PRCA, aiming to represent the clinical and flow cytometry characteristics of these concomitant diseases, analyze the mechanism between diseases, and provide a clinical reference. |
format | Online Article Text |
id | pubmed-8615434 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
spelling | pubmed-86154342021-11-26 CB-LPD, MGUS, T-LGLL, and PRCA: A rare case report of 4 concomitant hematological disorders Xu, Qinhong Yu, Jieni Lin, Xiaoyan Li, Youli Zhang, Kejie Medicine (Baltimore) 4800 RATIONALE: Monoclonal gammopathy of undetermined significance (MGUS) is a clinically asymptomatic clonal plasma cell or lymphoplasmacytic proliferative disorder. Recently, some case reports have described the association of pure red cell aplasia (PRCA) with MGUS, even with a relatively low monoclonal immunoglobulin burden. T large granular lymphocyte leukemia (T-LGLL) is a chronic lymphoproliferative disorder characterized by clonal expansion of T large granular lymphocytes, which is rare in China. There are some reports about T-LGL leukemia in patients with B-cell lymphoma; however, it is very rare that T-LGLL coexists with MGUS and clonal B-cell lymphoproliferative disorders (CB-LPD). PATIENT CONCERNS: A 77-year-old man was hospitalized because of anemia. He was diagnosed with MGUS, CB-LPD, and PRCA. During the development of the disease, a group of abnormal T lymphocytes was detected by flow cytometry of peripheral blood. DIAGNOSIS: Combining clinical manifestations with the result of T cell receptor gene rearrangement and immunophenotype, it was consistent with the diagnosis of T large granular lymphocyte leukemia. INTERVENTIONS: The patient was treat with bortezomib and dexamethasone regimen, Rituximab and sirolimus. OUTCOMES: The patient was transfusion independent after therapies. LESSONS: We report a patient with 4 concomitant hematological disorders: T-LGLL, MGUS, CB-LPD, and PRCA, aiming to represent the clinical and flow cytometry characteristics of these concomitant diseases, analyze the mechanism between diseases, and provide a clinical reference. Lippincott Williams & Wilkins 2021-11-24 /pmc/articles/PMC8615434/ /pubmed/34964755 http://dx.doi.org/10.1097/MD.0000000000027874 Text en Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 (https://creativecommons.org/licenses/by/4.0/) |
spellingShingle | 4800 Xu, Qinhong Yu, Jieni Lin, Xiaoyan Li, Youli Zhang, Kejie CB-LPD, MGUS, T-LGLL, and PRCA: A rare case report of 4 concomitant hematological disorders |
title | CB-LPD, MGUS, T-LGLL, and PRCA: A rare case report of 4 concomitant hematological disorders |
title_full | CB-LPD, MGUS, T-LGLL, and PRCA: A rare case report of 4 concomitant hematological disorders |
title_fullStr | CB-LPD, MGUS, T-LGLL, and PRCA: A rare case report of 4 concomitant hematological disorders |
title_full_unstemmed | CB-LPD, MGUS, T-LGLL, and PRCA: A rare case report of 4 concomitant hematological disorders |
title_short | CB-LPD, MGUS, T-LGLL, and PRCA: A rare case report of 4 concomitant hematological disorders |
title_sort | cb-lpd, mgus, t-lgll, and prca: a rare case report of 4 concomitant hematological disorders |
topic | 4800 |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8615434/ https://www.ncbi.nlm.nih.gov/pubmed/34964755 http://dx.doi.org/10.1097/MD.0000000000027874 |
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