Beyond HELLP: Peripartum Catastrophic Thromboembolism due to Hereditary Diffuse Gastric Cancer

Abstract

Peripartum thrombocytopenia and thrombotic events are usually attributable to diseases like Hemolysis Elevated Liver Enzymes Low Platelets (HELLP) and thrombotic thrombocytopenic purpura (TTP). Malignancy is a rare cause and gastric cancer even more unusual. We report the case of a 28-year-old female with undiagnosed hereditary diffuse gastric cancer (HDGC) in the peripartum period initially presenting with nausea and blurry vision at 35 weeks gestation. Following cesarean section 1 week later, she developed thrombocytopenia and elevated blood pressure; HELLP was diagnosed. Development of acute kidney injury, decreasing platelet count and anemia lead to consideration of TTP. Despite plasmapheresis she had no improvement, developed respiratory failure and had acute large vessel arterial and venous thrombosis with multiple cerebral, renal, splenic and lower extremity arterial infarctions. Deep venous thrombosis of lower limbs was also seen. She was placed on corticosteroids and anticoagulation. Following extensive workup, vegetations on mitral valve were seen on echocardiography and she was started on antibiotics for endocarditis. She deteriorated into refractory shock, multiple organ failure and died on postpartum day 8. Autopsy done revealed diffuse submucosal gastric adenocarcinoma (linitus plastica). Metastases were present on small bowel, fallopian tubes, ovary and the placenta. Mitral valve had marantic vegetations. Tumor cells showed CDH1 gene mutation. Such a diagnosis in pregnancy is very challenging due to overlapping symptoms leading to misdiagnosis. Here, thromboembolism was malignancy-related and rapid spread probably hormone-associated. The purpose of this report is to highlight the need to warrant investigation for cancer, among workup for other common conditions, for atypical thromboembolism in pregnancy.




J Clin Gynecol Obstet. 2018;7(3-4):81-83
doi: https://doi.org/10.14740/jcgo512w