关键词: 血栓性微血管病, 内皮功能, 肾脏病理

Abstract:

Abstract Objective：Investigate the clinical features, morphological characteristics, treatment and prognosis of the pregnancy-related thrombotic microangiopathy (TMA). Methodology: The clinical and pathological data from 11 pregnancy-related TMA patients were retrospectively analyzed from Jan, 2004 to April, 2014. Results: In total 11 patients which consisted with 2 thrombotic thrombocytopenic purpura (TTP) patients; 5 heomlyticuremicsyndrome (HUS) patients; 1 hemolysis, elevated liver enzymes, low platelet count syndrome (HELLP syndrome) patient; 3pre-eclampsia patients. The onset time was in the late of pregnancy or in one week of postpartum. All of the pregnant women were alive, but there were five cases of stillbirth. The typical clinical manifestations of patients were acute kidney injury, anemia and thrombocytopenia. There are different degrees of endothelial dysfunction in 11 patients. 8 patients received renal biopsy. The histological examination showed there were 7 patients with interstitial and blood vessel lesions, mainly involving the interlobular arteries, arterioles and afferent arterioles. The major lesions included fibrinoid necrosis, thrombosis, endothelial cell swelling, arterial "onion skin-like" change. All of the patients received continuous renal replacement therapy, 5 HUS patients received plasma exchange, and 2 TTP patients received plasma transfusion and immunosuppressive therapy. The renal function of the HUS and HELLP patients recovered completely, and the remaining patients come into the chronic renal insufficiency, two cases receiving maintenance hemodialysis treatment. Conclusions: Pregnancy-related TMA is a harmful disease which threatened the health of pregnant women and fetal life. Despite the molecular mechanisms of pregnancy-related TMA is difference, endothelial damage play a critical role. Distinguishing the cause of the disease, giving timely and effective individualized treatment is one of the important factors which affect the prognosis of pregnancy-related TMA.

Key words: thrombotic microangiopathy, endothelial function, renal pathology