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儿童非恶性淋巴增殖性疾病:病例系列

Non Malignant Lymphoproliferative Disorders in Children: A Case Series.

作者信息

Sivasankaran Meena, Bharadwaj Rishab, Sivabalan Somu, Thangadorai Ravikumar, Kumar Vimal, Munirathnam Deenadayalan

机构信息

Department of Paediatric Haemato-Oncology, Kanchi Kamakoti Childs Trust Hospital, 12A Nageswara Road, Numgambakkam, Chennai, 600 034 India.

Department of Paediatrics, Kanchi Kamakoti Childs Trust Hospital, Numgambakkam, Chennai, India.

出版信息

Indian J Hematol Blood Transfus. 2021 Jan;37(1):152-156. doi: 10.1007/s12288-020-01323-7. Epub 2020 Jul 27.

DOI:10.1007/s12288-020-01323-7
PMID:33707849
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7900327/
Abstract

Lymphoproliferative disorders occurs due to uncontrolled proliferation of lymphocytes that causes lymphocytosis, lymphadenopathy, and involvement of extra nodal sites (bone marrow, liver and spleen) and occur primarily due to immune dysfunction. We describe series of cases with non malignant LPD encountered in our practice and their varied clinical presentation, difficulties in diagnosis, underlying etiology, treatment and outcome. Many of these disorders are self limiting, however some are associated with significant morbidity, hence treatment must be tailored based on the underlying immune dysfunction and aggressiveness of the clone.

摘要

淋巴增殖性疾病是由于淋巴细胞不受控制地增殖导致淋巴细胞增多、淋巴结病以及结外部位(骨髓、肝脏和脾脏)受累而发生的,主要是由于免疫功能障碍引起的。我们描述了在我们的临床实践中遇到的一系列非恶性淋巴增殖性疾病病例及其多样的临床表现、诊断困难、潜在病因、治疗和预后。这些疾病中的许多是自限性的,然而有些与严重的发病率相关,因此治疗必须根据潜在的免疫功能障碍和克隆的侵袭性进行调整。

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Spectrum of Phenotypes Associated with Mutations in LRBA.与LRBA基因突变相关的表型谱
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