Swaminathan Neeraja, Sedhom Ramy, Shahzad Anum, Azmaiparashvili Zurab
Department of Internal Medicine, Albert Einstein Medical Center, Philadelphia, PA, USA.
J Community Hosp Intern Med Perspect. 2021 Mar 23;11(2):277-279. doi: 10.1080/20009666.2021.1883812.
This is a case of a 27-year-old primigravida with monochorionic diamniotic twin gestation who was admitted to the hospital for induction of labour. Her postpartum course was complicated by microangiopathic haemolytic anemia (MAHA). The etiology for the MAHA was initially thought to be secondary to pre-eclampsia and vitamin B12/folate deficiency. However, she had persistent anemia and further workup demonstrated that she had a left renal cell carcinoma (RCC) with perinephric haemorrhage consistent with Wunderlich syndrome. This case was intriguing because of its unusual presentation and the several diagnostic and therapeutic challenges along the way. : MAHA: microangiopathic haemolytic anaemia; RCC: renal cell carcinoma; BP: blood pressure; WS: Wunderlich syndrome; CT: computed tomography; LFTs: liver function tests; LDH: lactate dehydrogenase; HELLP: haemolysis elevated liver enzymes, low platelets; DIC: disseminated intravascular coagulation; PLASMIC: score for TTP - includes platelet count <30 x 109/L, evidence of haemolysis (reticulocyte count >2.5%, haptoglobin undetectable, or indirect bilirubin >2mg/dL), active cancer, history of solid organ transplant, mean corpuscular volume (MCV) <90fL, INR <1.5, creatinine <2mg/dL. Each item is sored as being present (YES) or not (NO). Absence of active cancer and solid organ transplant gets scored with a point each. The total points are added up to categorize the severity and risk of TTP. Low risk <4, Intermediate 5, high risk >6; TTP: thrombotic thrombocytopenic purpura; APLA- anti-phophospholipid antibody; BMI: body mass index; TMAs: thrombotic microangiopathies; HUS: haemolytic uremic syndrome; vWF: von Willebrand factor.
这是一例27岁初产妇,单绒毛膜双羊膜囊双胎妊娠,因引产入院。她产后病程并发微血管病性溶血性贫血(MAHA)。MAHA的病因最初被认为继发于子痫前期和维生素B12/叶酸缺乏。然而,她贫血持续存在,进一步检查显示她患有左肾细胞癌(RCC),伴有肾周出血,符合温德利希综合征(WS)。该病例因其不寻常的表现以及在此过程中出现的几个诊断和治疗挑战而引人关注。:MAHA:微血管病性溶血性贫血;RCC:肾细胞癌;BP:血压;WS:温德利希综合征;CT:计算机断层扫描;LFTs:肝功能检查;LDH:乳酸脱氢酶;HELLP:溶血、肝酶升高、血小板减少;DIC:弥散性血管内凝血;PLASMIC:血栓性血小板减少性紫癜评分 - 包括血小板计数<30×10⁹/L、溶血证据(网织红细胞计数>2.5%、触珠蛋白检测不到或间接胆红素>2mg/dL)、活动性癌症、实体器官移植史、平均红细胞体积(MCV)<90fL、国际标准化比值(INR)<1.5、肌酐<2mg/dL。每个项目根据存在(是)或不存在(否)进行评分。无活动性癌症和实体器官移植各得1分。将总分相加以对血栓性血小板减少性紫癜(TTP)的严重程度和风险进行分类。低风险<4分,中度5分,高风险>6分;TTP:血栓性血小板减少性紫癜;APLA - 抗磷脂抗体;BMI:体重指数;TMAs:血栓性微血管病;HUS:溶血性尿毒症综合征;vWF:血管性血友病因子