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血小板“贮存池病”。与多发性先天性海绵状血管瘤相关。

'Storage pool disease' of platelets. Association with multiple congenital cavernous hemangiomas.

作者信息

Khurana M S, Lian E C, Harkness D R

出版信息

JAMA. 1980 Jul 11;244(2):169-71.

PMID:7382076
Abstract

A women with congenital cavernous hemangiomas and a bleeding diathesis since childhood was found to have a qualitative platelet defect characterized by the absence of a second-phase aggregation induced by epinephrine, adenosine diphosphate (ADP), and collagen, accompanied by decreased levels of adenosine triphosphate (ATP) and ADP, with a high ATP-ADP ratio consistent with the diagnosis of "storage pool disease" of the platelets. There was no evidence of disseminated intravascular coagulation or circulating antiplatelet antibodies. The bleeding tendency responded to platelet transfusion.

摘要

一名患有先天性海绵状血管瘤且自幼有出血倾向的女性,被发现存在定性血小板缺陷,其特征为肾上腺素、二磷酸腺苷(ADP)和胶原诱导的第二相聚集缺失,同时伴有三磷酸腺苷(ATP)和ADP水平降低,ATP - ADP比值升高,符合血小板“储存池病”的诊断。没有弥散性血管内凝血或循环抗血小板抗体的证据。出血倾向对血小板输注有反应。

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