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全血细胞减少的一种罕见病因:惠普尔病。

An unusual cause of pancytopenia: Whipple's disease.

作者信息

Tun Nay T, Shukla Shwetanshu, Krishnakurup Jaykrishnan, Pappachen Binu, Krishnamurthy Mahesh, Salib Hayman

机构信息

Department of Internal Medicine, School of Medicine, Easton Hospital, Residency Program, Affiliated with Drexel University, Easton, PA, USA.

Hematology and Oncology Department, School of Medicine, Easton Hospital, Easton, PA, USA.

出版信息

J Community Hosp Intern Med Perspect. 2014 Apr 14;4(2). doi: 10.3402/jchimp.v4.23482. eCollection 2014.

Abstract

Whipple's disease is a systemic infectious disease caused by the bacteria Tropheryma whipplei. The most common clinical manifestations of Whipple's disease are weight loss (92%), hypoalbuminemia and steatorrhea (91%, respectively), diarrhea (72%), arthralgia (67%), and abdominal pain (55%). Neurological signs and symptoms from dementia to oculomasticatory myorhythmia or oculofacioskeletal myorhythmia (pathognomonic of Whipple's disease), lymphadenopathy, and fatigue can also be present. Pancytopenia is a rare and less recognized clinical feature in Whipple's disease patients. We are describing a case where a middle-aged Caucasian male diagnosed with Whipple's disease was found to have pancytopenia. Etiology of pancytopenia is postulated to be due to the invasion of bone marrow by T. whipplei. It is important to recognize that bone marrow involvement by the Whipple bacillus is not uncommon. In the presence of lymphadenopathy and pancytopenia, clinicians should think of Whipple's disease as a differential diagnosis apart from lymphoma or other non-specific granulomatous reticuloendothelial disorders.

摘要

惠普尔病是一种由惠普尔嗜组织菌引起的全身性传染病。惠普尔病最常见的临床表现为体重减轻(92%)、低白蛋白血症和脂肪泻(分别为91%)、腹泻(72%)、关节痛(67%)以及腹痛(55%)。还可能出现从痴呆到动眼咀嚼肌阵挛或动眼面骨肌阵挛(惠普尔病的特征性表现)、淋巴结病和疲劳等神经学症状和体征。全血细胞减少是惠普尔病患者中一种罕见且较少被认识到的临床特征。我们正在描述一例被诊断为惠普尔病的中年白种男性出现全血细胞减少的病例。全血细胞减少的病因推测是由于惠普尔嗜组织菌侵袭骨髓。必须认识到,惠普尔杆菌累及骨髓并不罕见。在存在淋巴结病和全血细胞减少的情况下,临床医生应将惠普尔病视为除淋巴瘤或其他非特异性肉芽肿性网状内皮系统疾病之外的鉴别诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8dc0/3992356/177cf5e93255/JCHIMP-4-23482-g001.jpg

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