Atypical hemolytic uremic syndrome: when pregnancy leads to lifelong dialysis: a case report and literature review

Abstract

Atypical hemolytic uremic syndrome (aHUS), a challenging disorder, commonly caused by inherited defects or regulatory processes of the complement alternative pathway. There are multiple causes, including pregnancy. Pregnancy provokes life-threatening episodes, preeclampsia, hemolysis elevated liver enzymes low platelets, microangiopathic hemolytic anemia (MAHA) and end-stage renal disease. Additionally, complement dysregulation and, with aHUS, affects fetal and maternal outcomes. Pregnancy-associated aHUS results in a poor prognosis with irreversible renal damage. Likewise, it is imperative to know that MAHA can provoke endothelial disruption, destruction of red cells and thrombocytopenia. We present a case of a young 18-year-old woman with MAHA and aHUS, requiring emergent cesarean section at 34 weeks of gestation and hemodialysis, secondary to complications from a recent pregnancy. Elevated blood pressure readings, rising creatinine levels, as well as her mother being on dialysis after pregnancy raised suspicion for thrombotic microangiopathy and aHUS. She was subsequently managed with plasma exchange, steroids, eculizumab and hemodialysis. Thus, plasma exchange should be initiated, with pending additional workup. Upon a definitive diagnosis of aHUS, eculizumab would be warranted to mitigate immune dysregulation. Understanding thrombotic microangiopathies diagnosis, and recognizing concomitant consequences, is vital. Having better insights into endothelial injuries can prevent unfortunate outcomes.

A Rare Case of HELLP Syndrome with Hematomas of Spleen and Liver, Eclampsia, Severe Hypertension and Prolonged Coagulopathy—A Case Report

“…Risk factors for HELLP are similar to those for other PIH forms [19][20][21][22]; (2) Prodromal symptoms of HELLP are nonspecific (malaise, nausea, epigastric pain, headache or visual symptoms) [3,23] but these prodromal symptoms may not be present either [2,3]; (3) Importantly, HELLP syndrome belongs to a broad spectrum of thrombotic microangiopathies (TMAs) characterized by hemolytic anemia, thrombocytopenia and organ damage as a result of microclots. Differentiation with other TMAs have important therapeutic implications [16,18,24,25].…”

“…Although currently less tested, the presence of schistiocytes in the blood smear tests and an increase in the number of reticulocytes, spherocytes and polychromatophilic erythrocytes confirm the hemolysis [18] However, this case demonstrates that thrombocytopenia in pregnancy urgently requires further diagnosis and our case drew attention to this. The HELLP syndrome must be differentiated from other thrombotic microangiopathies (TMAs) characterized by hemolysis (and microangiopathic hemolytic anemia), thrombocytopenia and damage to various end organs (especially kidneys) due to endothelial damage and microclots [18,24,25].…”

“…Blood clots occur in the tiny blood vessels (platelets aggregate), and the result is organ damage. In the HELLP syndrome, the liver is the major organ damaged, but the kidneys are often (>50% of HELLP cases) damaged as well [18,25,26,30,31]. It is possible that endovascular deposition of fibrin deposits (not connected with disseminated intravascular coagulation, DIC) limits blood flow through the liver sinusoids, and hypoxia leads to the formation of focal infarcts, subcapsular hematomas and interstitial hemorrhage [7,9,14,32].…”

“…The HELLP syndrome must be differentiated from TMAs, such as: thrombotic thrombocytopenic purpura (TTP); typical hemolytic uremic syndrome (HUS); and atypical hemolytic uremic syndrome (a-HUS) [24][25][26].…”

“…Deficiency of ADAMTS13 results in the formation of unusually large activated multimers of von Willebrand factor (VWF) on endothelial cells and the aggregation of platelets. This leads to blood clots in the small vessels and to hemolysis [18,24,25,33]. (2) Typical hemolytic uremic syndrome (HUS) is caused by a bacterial infection (mainly Escherichia coli) producing the Shiga toxin (and is abbreviated as STEC-HUS).…”