一种以门静脉海绵样变性为表现的特发性血小板增多症的潜在形式。
A latent form of essential thrombocythemia presenting as portal cavernoma.
机构信息
Department of General Surgery, Sir Run Run Shaw Hospital, College of Medicine, Sir Run Run Shaw Institute of Clinical Medicine of Zhejiang University, Hangzhou 310016, Zhejiang Province, China.
出版信息
World J Gastroenterol. 2009 Nov 14;15(42):5368-70. doi: 10.3748/wjg.15.5368.
Abstract
Essential thrombocythemia is frequently associated with abdominal thrombotic complications including portal cavernoma as a consequence of chronic portal vein thrombosis. Essential thrombocythemia in a latent form is difficult to identify at onset due to the absence of an overt disease phenotype. In the presented case report, the diagnosis of essential thrombocythemia was initially missed because the typical disease phenotype was masked by bleeding and hypersplenism. The correct diagnosis was only reached when the patient experienced persistent thrombocytosis and pseudohyperkalemia after a shunt operation.
摘要
原发性血小板增多症常伴有腹部血栓并发症，包括门静脉海绵样变，这是由于慢性门静脉血栓形成所致。原发性血小板增多症在潜伏期时由于缺乏明显的疾病表型，因此很难在发病时被识别。在本病例报告中，由于出血和脾功能亢进掩盖了典型的疾病表型，最初误诊为原发性血小板增多症。只有当患者在分流手术后出现持续性血小板增多和假性高钾血症时，才做出正确的诊断。
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