Case series of pregnancy-associated thrombotic microangiopathy diagnosed as postpartum fulminant HELLP syndrome and atypical hemolytic uremic syndrome

 

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Case series of pregnancy-associated thrombotic microangiopathy diagnosed as postpartum fulminant HELLP syndrome and atypical hemolytic uremic syndrome

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Tomoyuki
Otsuka
Tomoyuki Otsuka to-otsuka@juntendo.ac.jp Juntendo University Urayasu Hospital Nephrology Chiba Japan *
Yuya Sasatsuki y.sasatsuki.ct@juntendo.ac.jp Juntendo University Urayasu Hospital Nephrology Chiba Japan -
Mikoto Fujishiro m.fujishiro.ud@juntendo.ac.jp Juntendo University Urayasu Hospital Nephrology Chiba Japan -
Mai Akimoto m.akimoto.fg@juntendo.ac.jp Juntendo University Urayasu Hospital Nephrology Chiba Japan -
Yukako Umezawa y-umezawa@juntendo.ac.jp Juntendo University Urayasu Hospital Nephrology Chiba Japan -
Maiko Nakayama m-nakayama@juntendo.ac.jp Juntendo University Urayasu Hospital Nephrology Chiba Japan -
Masahiro Muto mmutou@juntendo.ac.jp Juntendo University Urayasu Hospital Nephrology Chiba Japan -
Hisatsugu Takahara h-taka@juntendo.ac.jp Juntendo University Urayasu Hospital Nephrology Chiba Japan -
Yusuke Suzuki shitoshi-uab@hotmail.com Juntendo university Faculty of Medicine Nephrology Tokyo Japan -
Hitoshi Suzuki shitoshi@juntendo.ac.jp Juntendo University Urayasu Hospital Nephrology Chiba Japan -
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Pregnancy-associated thrombotic microangiopathy (TMA) occurs in 1 in 25,000 births, accounting for 8-18% of all TMA cases. It is necessary to differentiate from thrombotic thrombocytopenic purpura, HELLP syndrome, anti-phospholipid antibody syndrome, atypical hemolytic uremic syndrome (aHUS). However, because these pathogenesis are similar, differentiation is often difficult. Pregnancy-associated TMA can be fatal if it becomes severe, thus early diagnosis and treatment intervention are essential.

 

Case 1: A 36-year-old female, G1P0, was admitted at 37 weeks gestation due to persistent nausea. Following a failed induction of labor, an emergency Cesarean section was performed. On postoperative day 3, she developed dyspnea along with substantial liver dysfunction, thrombocytopenia, and renal dysfunction, leading to a diagnosis of postpartum fulminant HELLP syndrome. She was successfully treated with plasma exchange (PE) and steroids, achieving improvement in hepatic and renal function, and making a favorable recovery.

Case 2: A 28-year-old female, G1P0, underwent an emergency Cesarean section at 32 weeks of gestation due to placental abruption. Postoperatively, she developed renal dysfunction, thrombocytopenia, and anemia. She presented with oliguria and fluid overload and required the initiation of continuous hemodiafiltration. Given the rapid progression of severe thrombocytopenia and kidney injuries, atypical hemolytic uremic syndrome (aHUS) was clinically diagnosed. Treatment with PE and the complement inhibitor eculizumab was initiated immediately. Subsequently, the platelet counts and kidney injuries were improved, allowing for the discontinuation of hemodialysis.

We encountered two cases of pregnancy-associated TMA. Careful differential diagnosis is essential in pregnancy-associated TMA, especially HELLP syndrome, thrombotic thrombocytopenic purpura, and aHUS. As the severe forms can be life-threatening, early diagnosis and prompt therapeutic intervention are crucial. 

Kewords