Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Xu, Qinhong, Yu, Jieni, Lin, Xiaoyan, Li, Youli, Zhang, Kejie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2021
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
_version_ 1784604103903543296
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
work_keys_str_mv AT xuqinhong cblpdmgustlgllandprcaararecasereportof4concomitanthematologicaldisorders
AT yujieni cblpdmgustlgllandprcaararecasereportof4concomitanthematologicaldisorders
AT linxiaoyan cblpdmgustlgllandprcaararecasereportof4concomitanthematologicaldisorders
AT liyouli cblpdmgustlgllandprcaararecasereportof4concomitanthematologicaldisorders
AT zhangkejie cblpdmgustlgllandprcaararecasereportof4concomitanthematologicaldisorders