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抗磷脂综合征中的骨髓坏死

Bone marrow necrosis in antiphospholipid syndrome.

作者信息

Paydas S, Koçak R, Zorludemir S, Baslamisli F

机构信息

Department of Oncology, Cukurova University Faculty of Medicine, Balcali, Adana, Turkey.

出版信息

J Clin Pathol. 1997 Mar;50(3):261-2. doi: 10.1136/jcp.50.3.261.

Abstract

Bone marrow necrosis (BMN) is a relatively rare entity and has been associated with a poor prognosis. It is most commonly found in patients with neoplastic disorders, severe infections and sickle cell anemia. An unusual case of antiphospholipid syndrome (APS) with extensive bone marrow necrosis is described in a 27 year old woman. The patient presented with severe pancytopenia, some cognitive impairment resulting from a previous cerebrovascular accident, fever, hypertension, dyspnoea, tachycardia, hepatosplenomegaly, and vaginal bleeding. Her laboratory findings included a strongly positive Coombs' test (anti-IgG and anti-C3d), a prothrombin time of 23 seconds and an activated partial thromboplastin time of 45 seconds. Anticardiolipin antibody tests were positive. Antinuclear and anti-DNA antibodies were negative but the anti-SM test was positive. A bone marrow biopsy specimen was reported as showing extensive necrosis. The patient was treated with steroids, transfusion, and plasma exchange with some clinical improvement but her pancytopenia did not respond and necessitated frequent transfusions. This case lends further support to the association between APS and BMN.

摘要

骨髓坏死(BMN)是一种相对罕见的病症,且与预后不良相关。它最常见于患有肿瘤性疾病、严重感染和镰状细胞贫血的患者中。本文描述了一名27岁女性患抗磷脂综合征(APS)并伴有广泛骨髓坏死的罕见病例。该患者表现为严重全血细胞减少、既往脑血管意外导致的一些认知障碍、发热、高血压、呼吸困难、心动过速、肝脾肿大以及阴道出血。她的实验室检查结果包括库姆斯试验(抗IgG和抗C3d)呈强阳性、凝血酶原时间为23秒以及活化部分凝血活酶时间为45秒。抗心磷脂抗体检测呈阳性。抗核抗体和抗DNA抗体为阴性,但抗SM试验呈阳性。一份骨髓活检标本报告显示存在广泛坏死。该患者接受了类固醇、输血及血浆置换治疗,临床症状有所改善,但全血细胞减少未见好转,需要频繁输血。此病例进一步支持了APS与BMN之间的关联。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7de5/499827/064c82e166d1/jclinpath00252-0084-a.jpg

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