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窦状隙阻塞综合征。

Sinusoidal obstruction syndrome.

机构信息

Department of Hepatology and INSERM U1053, Haut-Lévêque Hospital, University Hospital of Bordeaux, avenue Magellan, 33600 Pessac, France.

Department of Hematology, Bretonneau Hospital, University hospital of Tours, 2, boulevard Tonnellé, 37000 Tours, France.

出版信息

Clin Res Hepatol Gastroenterol. 2020 Sep;44(4):480-485. doi: 10.1016/j.clinre.2020.03.019. Epub 2020 Apr 3.

DOI:10.1016/j.clinre.2020.03.019
PMID:32253162
Abstract

Sinusoidal obstruction syndrome (SOS), previously known as veno-occlusive disease, is characterized by concentric and non-thrombotic obstruction of the sinusoid and central vein lumen with no identified primitive or thrombotic hepatic vein lesions. The initial lesion is a result of endothelial denudation, corresponding to the migration of damaged sinusoidal cells to the central veins of the hepatic lobules, leading to sinusoidal and veno-occlusive congestive obstruction. SOS may be associated with other lesions such as centrilobular perisinusoidal fibrosis, peliosis, or nodular regenerative hyperplasia. The first cases of SOS were documented in 1920 in South Africa, after ingestion of food sources contaminated by pyrrolizidine alkaloids. SOS is a well-known complication of hematopoietic stem cell transplantation (HSCT). Numerous toxins and drugs have been associated with SOS, mainly chemotherapies and immunosuppressive therapies, as well as total body or liver irradiation and ABO mismatch platelet transfusion. The pathogenesis of this entity remains unknown.

摘要

窦状隙阻塞综合征(SOS),以前称为静脉阻塞性疾病,其特征为窦状隙和中央静脉管腔呈同心性且非血栓性阻塞,并无明确的肝原始静脉或血栓性静脉病变。最初的病变是内皮细胞脱落的结果,对应于受损的窦状细胞迁移到肝小叶的中央静脉,导致窦状隙和静脉阻塞性充血性阻塞。SOS 可能与其他病变相关,如中央静脉周围纤维变性、血窦扩张或结节性再生性增生。SOS 的首例病例于 1920 年在南非记录,是由于摄入了受吡咯里西啶生物碱污染的食物源。SOS 是造血干细胞移植(HSCT)的一种熟知的并发症。许多毒素和药物与 SOS 相关,主要是化疗和免疫抑制疗法,以及全身或肝脏照射和 ABO 不匹配血小板输注。该实体的发病机制仍不清楚。

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