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[脾脏囊性淋巴管瘤伴脾功能亢进]

[Cystic lymphangioma of the spleen associated with hypersplenism].

作者信息

Santagati G, Marra M, Scarlata S, Garbagna P, Luppino R, Forgione F

出版信息

Minerva Med. 1978 Nov 10;69(54):3705-13.

PMID:733052
Abstract

A rare case of cystic lymphangioma of the spleen accompanying hypersplenism syndrome is described. After stressing the importance of certain examinations such as arteriography and splenic scintigraphy in diagnosis the condition, the many ways in which cystic lymphangioma of the spleen may present clinically are reviewed; these go from asymptomatic to straightforward splenomegaly, with or without clinical signs, up to a clear-cut hypersplenism syndrome. The present case merits consideration for three reasons: 1) The absolutely exceptional nature of cystic lymphangioma of the spleen; 2) Exceptional association with hypersplenism syndrome; 3) Remission of the syndrome following splenectomy. It is concluded that, notwithstanding the condition's rarity, cystic lymphangioma of the spleen should be considered in the differential diagnosis of splenomegalic syndromes when these are also accompanied by hypersplenism.

摘要

本文描述了一例伴有脾功能亢进综合征的脾脏囊性淋巴管瘤罕见病例。在强调诸如动脉造影和脾闪烁扫描等特定检查在诊断该疾病中的重要性之后,回顾了脾脏囊性淋巴管瘤可能出现的多种临床症状;这些症状从无症状到单纯脾肿大,伴有或不伴有临床体征,直至明确的脾功能亢进综合征。本病例值得关注有三个原因:1)脾脏囊性淋巴管瘤的绝对罕见性;2)与脾功能亢进综合征的罕见关联;3)脾切除术后综合征缓解。得出的结论是,尽管该疾病罕见,但当脾肿大综合征伴有脾功能亢进时,在鉴别诊断中应考虑脾脏囊性淋巴管瘤。

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