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青年急性肢体缺血:原发性血小板增多症的罕见病例

Acute Limb Ischemia in the Young: A Rare Case of Essential Thrombocytosis.

作者信息

Nugroho Johanes, Gunadi Ruth

机构信息

Department of Cardiology and Vascular Medicine, Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia.

Dr. Soetomo General Hospital, Surabaya, Indonesia.

出版信息

Case Rep Cardiol. 2021 Nov 20;2021:2563578. doi: 10.1155/2021/2563578. eCollection 2021.

Abstract

Acute limb ischemia (ALI) is rarely observed in young populations. The hypercoagulable state is a notable cause of ALI other than artery disease progression and cardiac embolization. A hypercoagulable state occurs in essential thrombocytosis because of the overproduction of hematopoietic cells secondary to the mutation of the JAK2, CALR, or MPL genes. We report a rare case of a 37-year-old woman presenting with Rutherford IIA ALI in the left lower extremity. Laboratory data revealed she had a platelet count reaching up to 1.38 mil/L, with other blood profiles being normal. A JAK2 mutation examination was later performed and proved positive. After careful management with catheter-directed thrombolysis, surgical thrombectomy, and cytoreductive therapy using hydroxyurea, the symptoms subsided and eventually restored the patient to physical activity in less than one month.

摘要

急性肢体缺血(ALI)在年轻人群中很少见。高凝状态是除动脉疾病进展和心脏栓塞之外导致ALI的一个显著原因。由于JAK2、CALR或MPL基因突变导致造血细胞过度生成,原发性血小板增多症会出现高凝状态。我们报告一例罕见病例,一名37岁女性出现左下肢卢瑟福IIA级ALI。实验室数据显示她的血小板计数高达138万/升,其他血液指标正常。后来进行了JAK2突变检测,结果呈阳性。经过导管定向溶栓、手术取栓以及使用羟基脲进行减细胞治疗的精心处理后,症状消退,最终患者在不到一个月的时间内恢复了体力活动。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c03f/8627342/0bea3a3e93c5/CRIC2021-2563578.001.jpg

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