Department of Hepatology, Osaka City General Hospital, 2-13-22, Miyakojima-hondori, Miyakojima-ku, Osaka, 534-0021, Japan.
Department of Gastroenterology, Osaka City General Hospital, 2-13-22, Miyakojima-hondori, Miyakojima-ku, Osaka, 534-0021, Japan.
Clin J Gastroenterol. 2022 Oct;15(5):907-912. doi: 10.1007/s12328-022-01673-x. Epub 2022 Jul 13.
Polycythemia vera is a chronic myeloproliferative disorder characterized by clonal proliferation of bone marrow progenitors. We report a case of ischemic enteritis and bowel obstruction resulting from polycythemia vera. A 76-year-old man was admitted to our hospital with abdominal distention. Contrast-enhanced computed tomography revealed portal vein thrombosis, superior mesenteric vein thrombosis, and dilated small intestinal loops with caliber changes at the end of the ileum. Laboratory data on admission revealed increased leukocyte, red blood cell, hemoglobin, and platelet levels. Polymerase chain reaction analysis for Janus kinase 2 V617F point mutation was positive. Intestinal obstruction due to either bowel adhesion or paralytic ileus secondary to the superior mesenteric artery and vein thrombosis caused by polycythemia vera was diagnosed. For decompression of the small intestinal obstruction, a transnasal ileus tube was placed. Despite conservative therapy, the small intestinal obstruction did not improve remarkably. Therefore, we decided to perform surgical treatment. Operative findings revealed extensive stricture in the ileum. Altogether, 30 cm of the ileum, including the known intestinal strictures, was resected. The pathological findings were consistent with ischemic enteritis. To the best of our knowledge, no case of ischemic enteritis caused by polycythemia vera has been previously reported in the literature.
真性红细胞增多症是一种以骨髓祖细胞克隆性增殖为特征的慢性骨髓增殖性疾病。我们报告了一例由真性红细胞增多症引起的缺血性肠炎和肠梗阻。一名 76 岁男性因腹胀入院。增强 CT 显示门静脉血栓形成、肠系膜上静脉血栓形成和末端回肠的小肠环扩张伴口径改变。入院时的实验室数据显示白细胞、红细胞、血红蛋白和血小板水平升高。Janus 激酶 2 V617F 点突变的聚合酶链反应分析呈阳性。诊断为真性红细胞增多症引起的肠系膜上动脉和静脉血栓形成导致肠粘连或麻痹性肠梗阻引起的肠梗阻。为了缓解小肠梗阻，放置了经鼻肠梗阻管。尽管进行了保守治疗，但小肠梗阻并未明显改善。因此，我们决定进行手术治疗。手术发现回肠广泛狭窄。总共切除了包括已知肠狭窄在内的 30cm 的回肠。病理结果符合缺血性肠炎。据我们所知，文献中尚无真性红细胞增多症引起的缺血性肠炎的报道。
