自身免疫性溶血性贫血伴蛋白C缺乏症的脾梗死1例

The Korean Journal of Hematology Pub Date : 2011-12-01 Epub Date: 2011-12-27 DOI:10.5045/kjh.2011.46.4.274
Min Yong Park, Jung A Kim, Seong Yoon Yi, Sun Hee Chang, Tae Hyun Um, Hye Ran Lee
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引用次数: 8

摘要

脾梗死最常由心血管血栓栓塞引起;然而,脾梗死也可发生在血液学疾病,包括镰状细胞病、遗传性球形细胞增多症、慢性骨髓增生性疾病、白血病和淋巴瘤。虽然10%的脾梗死是由血液系统疾病引起的,但很少伴有自身免疫性溶血性贫血(AIHA)。我们报告一例47岁女性缺铁性贫血,表现为左上腹部疼痛,并被诊断为AIHA和脾梗死。蛋白C活性和抗原活性分别降至44.0%(60 ~ 140%)和42.0%(65 ~ 140%)。实验室检查证实没有临床原因导致蛋白C缺乏,如弥散性血管内凝血、败血症、肝功能障碍或急性呼吸窘迫综合征。蛋白C缺乏伴脾梗死在病毒感染、遗传性球形红细胞增多症和白血病患者中有报道。这是一例罕见的AIHA患者脾梗死和短暂性蛋白C缺乏的病例。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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

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

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

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

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.

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