Ameda Saki, Kuroda Hiroyuki, Yamada Michiko, Sato Ken, Miura Shogo, Sakano Hiroya, Shibata Takanori, Uemura Naoki, Abe Tomoyuki, Fujii Shigeyuki, Maeda Masahiro, Fujita Miri, Kobune Masayoshi, Kato Junji
Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital.
Dept. of Pathology and Clinical Laboratory, Steel Memorial Muroran Hospital.
Rinsho Ketsueki. 2017;58(6):637-642. doi: 10.11406/rinketsu.58.637.
A 47-year-old man presented at a local ophthalmological hospital with blurred vision. He had been diagnosed with hypertensive retinopathy and renal failure and was referred to our hospital for treatment. A renal biopsy was done to evaluate pathology of high proteinuria, hematuria, and rapidly progressive glomerulonephritis. Blood pressure remained high despite antihypertensive therapy; anemia and thrombocytopenia gradually progressed. Thrombotic microangiopathy (TMA) was suspected based on red blood cell fragmentation due to hemolytic anemia, thrombocytopenia, and renal failure. However, plasma exchange resolved neither thrombocytopenia nor renal failure, and anemia gradually progressed. Backache suddenly developed 13 days later, and CT findings indicated a retroperitoneal hematoma secondary to bleeding from the kidney. Selective renal artery embolization via angiography stopped the bleeding, but the patient went into hemorrhagic shock. Pathological findings on renal biopsy were identical to those in malignant hypertension, namely an edematous membrane lining, thickened arterioles, and stenosis. We diagnosed thrombotic microangiopathy due to malignant hypertension, without decrease in activities of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif) or its antibodies. Renal failure did not improve, and continuous hemodiafiltration was needed. This procedure stabilized blood pressure and improved the TMA.
一名47岁男性因视力模糊就诊于当地眼科医院。他此前被诊断为高血压性视网膜病变和肾衰竭,随后被转诊至我院接受治疗。进行了肾活检以评估高蛋白尿、血尿和快速进展性肾小球肾炎的病理情况。尽管接受了抗高血压治疗,血压仍居高不下;贫血和血小板减少症逐渐加重。基于溶血性贫血导致的红细胞碎片、血小板减少症和肾衰竭,怀疑为血栓性微血管病(TMA)。然而,血浆置换既未缓解血小板减少症,也未改善肾衰竭,贫血仍逐渐加重。13天后突然出现背痛,CT检查结果显示为肾出血继发的腹膜后血肿。通过血管造影进行选择性肾动脉栓塞止住了出血,但患者陷入失血性休克。肾活检的病理结果与恶性高血压相同,即内膜水肿、小动脉增厚和狭窄。我们诊断为恶性高血压所致的血栓性微血管病,ADAMTS13(含Ⅰ型血小板反应蛋白基序的解聚素和金属蛋白酶)活性及其抗体均无降低。肾衰竭未改善,需要持续进行血液透析滤过。该治疗稳定了血压并改善了血栓性微血管病。