Abstract Introduction Hemoglobin SC (HbSC) disease is a combination of sickle cell trait and hemoglobin C (HbC). HbSC results in decreased red blood cell (RBC) solubility and life span. In the setting of infection, premature erythrocyte destruction can increase the risk for bone marrow suppression, leading to severe anemia and end-organ failure. We present a unique case of newly diagnosed HbSC disease in an adult presenting as multi-organ failure. Case A 24-year-old man with reported sickle cell trait and a recent upper respiratory infection presented with minimal responsiveness and altered mental status. Severe hypoxemia prompted intubation upon admission. Chest X-ray showed bilateral opacities, concerning for acute chest syndrome. His preliminary infectious work-up was negative for bacterial and viral pneumonia. Given his acute encephalopathy, MRI of the brain was obtained which demonstrated innumerable foci of ischemia located along the splenium of the corpus callosum bilaterally. Complete blood cell count revealed low red blood cell count, hemoglobin, and concurrent thrombocytopenia. Further testing showed an increase in nucleated red blood cells and lactate dehydrogenase along with polychromasia, target cells, and sickle cells on peripheral smear. These findings were suggestive of a hemolytic sickle cell crisis with cerebrovascular thrombosis. Hemoglobin electrophoresis showed 44.4% HbA, 25.2% HbS and 21.5% HbC, resulting in the diagnosis of HbSC disease. Viral serology revealed significantly elevated IgG and IgM Parvovirus B19 antibodies, later identified as the likely trigger of this crisis. The patient underwent exchange transfusion and improved significantly before being extubated on hospital day 6 and discharged on day 12. Discussion Cytopenias and end-organ dysfunction are well documented in sickle cell disease (HbSS). However, there have been no cases to our knowledge of severe multi-organ failure secondary to Parvovirus B19 infection in an adult patient with HbSC. The increased concentration of HbS in this patient likely contributed to the severity of his disease. This case highlights the importance of considering HbSC variant as a disease that can have severe complications and present similarly to HbSS in the right clinical scenario. It also emphasizes the importance of reconsidering previously established “chart diagnoses” when that diagnosis does not explain the progression of disease, as prompt and appropriate treatment can be lifesaving. This abstract is funded by: None
Harkins et al. (Fri,) studied this question.