Case 1
A 29-weeks baby girl born to a mother with SARS-CoV-2 pneumonia and acute respiratory failure (Table 1) was delivered due to non-reassuring fetal heart tones. She had an uneventful hospital course until day of life (DOL) 17 when she developed abdominal distension, hypotension, and bloody stools. She underwent a sepsis evaluation, and antibiotics were started. The initial abdomen X-ray was negative for pneumatosis, and an abdominal ultrasound was negative for volvulus or malrotation. She received red blood cells (RBC) transfusion for hemoglobin of 10.1 g/dL. On DOL 18, her status deteriorated with respiratory failure requiring intubation, refractory hypotension requiring vasopressors, persistent bloody stools, and hemoglobinuria. A blood transfusion reaction was suspected and given minor crossmatch positive, T-cryptantigen (TCA) activation was considered likely. The peripheral blood smear reported anemia with moderate anisocytosis, nucleated RBC, and increased spherocytes and schistocytes. Plasma free hemoglobin was elevated to 2280 mg/dL. She also developed thrombocytopenia and coagulopathy. The patient received blood products that were less likely to exacerbate hemolysis: washed RBC and platelets, prothrombin complex concentrate (PCC), and fibrinogen concentrate with improvement in coagulopathy.
The clinical course worsened with multiorgan failure and she underwent an exploratory laparotomy where a large sigmoid perforation was found. Over the next two days, the baby underwent two additional explorations of the abdomen and entire small bowel and colon were found to be ischemic, with multiple focal areas of necrosis. After discussions with the family, the care was redirected.
The autopsy’s report was consistent with necrotizing enterocolitis totalis. Additionally, multiorgan microvascular fibrin thrombi involving heart, renal glomeruli, stomach, small and large bowel may have been associated with intravascular hemolysis.