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.