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Gastrointestinal Vasoocclusive Crisis in a Woman with Hemoglobin SC Disease

Published:January 09, 2022DOI:https://doi.org/10.1016/j.amjmed.2021.12.004
      A 32-year-old woman with hemoglobin sickle cell (SC) disease was admitted to the hospital at 33 weeks gestation with pain in her arms and legs, consistent with vasoocclusive crisis. She was hypertensive and had proteinuria. Her platelet count decreased to 46 K/μL, and aspartate aminotransferase increased to 344 units/L, alanine aminotransferase to 92 units/L, and lactate dehydrogenase to 11,141 units/L. Peripheral blood smear showed schistocytes, in addition to the sickled and target cells seen in hemoglobin SC disease (Figure 1). A diagnosis of hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome was made, and she underwent emergent caesarean delivery. She developed abdominal distention on postoperative day 1. On postoperative day 2, her platelet count and transaminases had improved, but she developed severe abdominal pain.
      Figure 1
      Figure 1Peripheral blood smear. (A, B) Schistocytes (black arrows) and nucleated red blood cells (white arrows). (C) A sickle cell (black arrow) next to a neutrophil with dark azurophilic cytoplasmic granules consistent with toxic granulations (white arrow). (D) A target cell (black arrow) next to a neutrophil with toxic granulations (white arrow).
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