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一名年轻男性出现持续性血小板减少伴脾肿大,脾切除术后血小板计数反弹增高,随后发生肺栓塞:脾边缘区细胞血管瘤及相关事件

Persistent thrombocytopaenia in a young man with splenomegaly, rebound thrombocytosis after splenectomy and subsequent pulmonary embolism: splenic littoral cell angioma and associated events.

作者信息

de Ridder Gustaaf Gregoire, Galeotti Jonathan, Carney John, Wang Endi

机构信息

Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA.

出版信息

BMJ Case Rep. 2015 Nov 24;2015:bcr2015212882. doi: 10.1136/bcr-2015-212882.

Abstract

Littoral cell angioma (LCA) is a rare endothelial cell neoplasm in the spleen. Although many cases of LCA are asymptomatic, some present with signs and symptoms related to splenomegaly, whereas others manifest with haematological abnormalities, including anaemia and/or thrombocytopaenia (ie, hypersplenism). We report a case of LCA presenting with chronic thrombocytopaenia, probably due to splenic sequestration of platelets or phagocytosis of platelets by neoplastic cells. Following therapeutic splenectomy, the patient suffered from a marked rebound thrombocytosis and subsequently developed pulmonary embolisms. He was treated with anticoagulant therapy combined with antiplatelet therapy, and his symptoms were quickly resolved. This case emphasises an exclusion of primary splenic disorders in patients with chronic thrombocytopaenia, especially in those with splenomegaly and the contemplation of thromboembolism prophylaxis postsplenectomy.

摘要

脾窦岸细胞血管瘤(LCA)是脾脏中一种罕见的内皮细胞瘤。尽管许多LCA病例无症状,但有些会出现与脾肿大相关的体征和症状,而另一些则表现为血液学异常,包括贫血和/或血小板减少(即脾功能亢进)。我们报告一例LCA表现为慢性血小板减少,可能是由于脾脏对血小板的扣押或肿瘤细胞对血小板的吞噬作用。治疗性脾切除术后,患者出现明显的反应性血小板增多症,随后发生肺栓塞。他接受了抗凝治疗联合抗血小板治疗,症状迅速缓解。该病例强调对于慢性血小板减少患者,尤其是伴有脾肿大的患者,要排除原发性脾脏疾病,并考虑脾切除术后的血栓栓塞预防。

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Case Rep Gastrointest Med. 2015;2015:474969. doi: 10.1155/2015/474969. Epub 2015 Jan 29.
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Vascular complications after splenectomy for hematologic disorders.血液系统疾病脾切除术后的血管并发症。
Blood. 2009 Oct 1;114(14):2861-8. doi: 10.1182/blood-2009-04-210112. Epub 2009 Jul 27.
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Postsplenectomy reactive thrombocytosis.脾切除术后反应性血小板增多症。
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