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由于假性血小板减少症导致 CD36 缺乏症患者的难治性血小板输注。

Refractory platelet transfusion in a patient with CD36 deficiency due to pseudothrombocytopenia.

机构信息

Department of Hematology, 303rd Hospital of the People's Liberation Army, Nanning, Guangxi, China.

出版信息

Platelets. 2011;22(3):237-40. doi: 10.3109/09537104.2010.533798. Epub 2010 Dec 14.

DOI:10.3109/09537104.2010.533798
PMID:21143025
Abstract

Type I CD36 deficiency is defined by the absence of CD36 on both platelets and monocytes. Pseudothrombocytopenia (PTCP) is characterized by a false reduction in the number of platelets in ethylenediaminetetraacetic acid (EDTA)-anticoagulated blood. Here we report a rare case of concomitant CD36 deficiency and PTCP. The patient was a 7-year-old boy who suffered comminuted fractures of the left humeral condyle. In the pre-operative examination, he was found to have thrombopenia and assumed to have idiopathic thrombocytopenic purpura. After immunotherapy and platelet transfusion, the platelet count remained low, suggesting that the patient was refractory to platelet transfusion. Serum was collected for the detection of platelet antibodies, and antibodies against CD36 were found. Flow cytometry verified the absence of CD36 on both the platelets and monocytes of this patient. However, the platelet count was normal when capillary blood smears were analysed; in addition, platelet coagulation was noted under the microscope when EDTA-anticoagulated peripheral blood was used. The patient underwent surgery without platelet transfusion and recovered uneventfully.

摘要

I 型 CD36 缺乏症的定义是血小板和单核细胞上均缺乏 CD36。假性血小板减少症(PTCP)的特征是在乙二胺四乙酸(EDTA)抗凝的血液中血小板数量假性减少。本文报告了一例罕见的同时存在 CD36 缺乏症和 PTCP 的病例。患者为 7 岁男孩,患有左肱骨髁粉碎性骨折。术前检查发现血小板减少,考虑为特发性血小板减少性紫癜。免疫治疗和血小板输注后,血小板计数仍较低,提示患者对血小板输注无效。采集血清用于检测血小板抗体,并发现针对 CD36 的抗体。流式细胞术验证了该患者的血小板和单核细胞均缺乏 CD36。然而,当分析毛细血管血涂片时,血小板计数正常;此外,当使用 EDTA 抗凝外周血时,在显微镜下观察到血小板凝聚。该患者在未进行血小板输注的情况下接受了手术,恢复顺利。

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