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一名自身免疫性溶血性贫血和蛋白C缺乏患者的脾梗死

Splenic infarction in a patient with autoimmune hemolytic anemia and protein C deficiency.

作者信息

Park Min Yong, Kim Jung A, Yi Seong Yoon, Chang Sun Hee, Um Tae Hyun, Lee Hye Ran

机构信息

Division of Hematology/Oncology, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang, Korea.

出版信息

Korean J Hematol. 2011 Dec;46(4):274-8. doi: 10.5045/kjh.2011.46.4.274. Epub 2011 Dec 27.

Abstract

Splenic infarction is most commonly caused by cardiovascular thromboembolism; however, splenic infarction can also occur in hematologic diseases, including sickle cell disease, hereditary spherocytosis, chronic myeloproliferative disease, leukemia, and lymphoma. Although 10% of splenic infarction is caused by hematologic diseases, it seldom accompanies autoimmune hemolytic anemia (AIHA). We report a case of a 47-year-old woman with iron deficiency anemia who presented with pain in the left upper abdominal quadrant, and was diagnosed with AIHA and splenic infarction. Protein C activity and antigen decreased to 44.0% (60-140%) and 42.0% (65-140%), respectively. Laboratory testing confirmed no clinical cause for protein C deficiency, such as disseminated intravascular coagulation, sepsis, hepatic dysfunction, or acute respiratory distress syndrome. Protein C deficiency with splenic infarction has been reported in patients with viral infection, hereditary spherocytosis, and leukemia. This is a rare case of splenic infarction and transient protein C deficiency in a patient with AIHA.

摘要

脾梗死最常见的病因是心血管血栓栓塞;然而,脾梗死也可发生于血液系统疾病,包括镰状细胞病、遗传性球形红细胞增多症、慢性骨髓增殖性疾病、白血病和淋巴瘤。虽然10%的脾梗死由血液系统疾病引起,但很少伴有自身免疫性溶血性贫血(AIHA)。我们报告一例47岁缺铁性贫血女性患者,该患者左上腹疼痛,被诊断为AIHA和脾梗死。蛋白C活性和抗原分别降至44.0%(60 - 140%)和42.0%(65 - 140%)。实验室检查证实不存在蛋白C缺乏的临床病因,如弥散性血管内凝血、脓毒症、肝功能障碍或急性呼吸窘迫综合征。病毒感染、遗传性球形红细胞增多症和白血病患者中曾有蛋白C缺乏伴脾梗死的报道。这是一例AIHA患者发生脾梗死和短暂性蛋白C缺乏的罕见病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd5c/3259520/b9255055f0a2/kjh-46-274-g001.jpg

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