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以中性粒细胞减少性脓毒症为表现的严重再生障碍性贫血

Severe Aplastic Anemia Presenting as Neutropenic Sepsis.

作者信息

Daniels Natasha Faye, Burrin Charlotte, Ridwan Raiiq

机构信息

Faculty of Medicine, University of Cambridge School of Clinical Medicine, Cambridge, UK.

Emergency Medicine, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

出版信息

J Med Cases. 2020 Nov;11(11):339-341. doi: 10.14740/jmc3570. Epub 2020 Sep 23.

DOI:10.14740/jmc3570
PMID:34434342
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8383499/
Abstract

A 40-year-old female with aplastic anemia presented with a gum abscess deteriorating into neutropenic sepsis. Infection is an incredibly rare initial manifestation of aplastic anemia even on a background of significant neutropenia, hence the uniqueness of this case. The patient's initial complaints were of a subacute history of heavy vaginal bleeding and unexplained bruising, however on examination in the emergency department the patient was also noted to be pyrexial with gingival hyperplasia and a left sided submandibular lymphadenopathy. Initial blood results were phoned through from the lab reporting pancytopenia, confirming clinical suspicion of neutropenic sepsis. Antibiotic therapy was commenced and maxillofacial review for her unrelenting jaw pain revealed a gum abscess ultimately requiring tooth extraction. The patient underwent bone marrow biopsy showing hypocellular marrow with erythroid-dominant, dysplastic hematopoiesis. A thorough panel of investigations to rule out secondary causes led to the diagnosis of aplastic anemia, for which the patient is currently being managed with oral ciclosporin plus eltrombopag-bridging therapy, plus counselling for the potential requirement for stem cell transplant.

摘要

一名40岁再生障碍性贫血女性患者,因牙龈脓肿恶化为中性粒细胞减少性败血症。即使在严重中性粒细胞减少的背景下,感染作为再生障碍性贫血的初始表现也极其罕见,因此该病例具有独特性。患者最初的主诉是有大量阴道出血和不明原因瘀伤的亚急性病史,然而在急诊科检查时,还发现患者发热,伴有牙龈增生和左侧颌下淋巴结病。实验室通过电话传来的初步血液检查结果报告全血细胞减少,证实了临床对中性粒细胞减少性败血症的怀疑。开始使用抗生素治疗,对其持续的颌部疼痛进行颌面检查发现牙龈脓肿,最终需要拔牙。患者接受了骨髓活检,显示骨髓细胞减少,以红系为主,造血异常。通过全面的一系列检查排除继发原因后,诊断为再生障碍性贫血,目前该患者正在接受口服环孢素加艾曲泊帕桥接治疗,并接受关于干细胞移植潜在需求的咨询。

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本文引用的文献

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Aplastic anemia: a common hematological abnormality among peripheral pancytopenia.再生障碍性贫血:外周全血细胞减少中常见的血液学异常。
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Hepatitis-associated aplastic anaemia: epidemiology and treatment results obtained in Europe. A report of The EBMT aplastic anaemia working party.肝炎相关性再生障碍性贫血:欧洲的流行病学和治疗结果。EBMT 再生障碍性贫血工作组报告。
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Blood. 1995 Jun 1;85(11):3058-65.