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产后噬血细胞性淋巴组织细胞增生症(HLH)与抗磷脂综合征(APS)的罕见关联:是否存在显著重叠?

Rare association of haemophagocytic lymphohistiocytosis (HLH) and antiphospholipid syndrome (APS) in postpartum period: a considerable overlap?

机构信息

Critical Care Medicine, St John's Medical College Hospital, Bangalore, Karnataka, India.

General Medicine, St John's Medical College Hospital, Bangalore, Karnataka, India

出版信息

BMJ Case Rep. 2022 Feb 2;15(2):e247376. doi: 10.1136/bcr-2021-247376.

DOI:10.1136/bcr-2021-247376
PMID:35110287
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8811553/
Abstract

Haemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening disease of disproportionate immune activation. We present a case of a 25-year-old woman who is postnatal day 26 status post lower segment caesarean section who came with fever, breathlessness and abdominal pain in shock in the background of a malarial infection 3 days ago, which was initially evaluated on the lines of fever with multiple organ dysfunction syndrome. On further evaluation turned out to fulfil the criteria for secondary HLH seemingly triggered by an infectious cause or an autoimmune phenomenon. On further assessment, she was found to be positive for antiphospholipid antibodies which forced other possible diagnoses such as catastrophic antiphospholipid antibody syndrome into contention.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)是一种严重的、危及生命的免疫激活失调性疾病。我们报告了 1 例 25 岁产后 26 天的女性病例,该患者因疟疾感染 3 天前出现发热、呼吸急促和腹痛,并伴有休克,最初按照发热伴多器官功能障碍综合征的原则进行评估。进一步评估后发现符合继发性 HLH 的标准,似乎由感染原因或自身免疫现象引发。进一步评估发现该患者抗磷脂抗体阳性,这使得其他可能的诊断,如灾难性抗磷脂抗体综合征,成为争论的焦点。

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Can we truly diagnose adult secondary hemophagocytic lymphohistiocytosis (HLH)? A critical review of current paradigms.我们能否真正诊断成人继发性噬血细胞性淋巴组织细胞增生症(HLH)?对当前范式的批判性回顾。
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