Post-partum occurrence of Wunderlich syndrome and microangiopathic haemolytic anaemia (MAHA): a case report.

ABSTRACT

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. Abbreviations 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.