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Polymerized human Hb use in acute chest syndrome: a case report
Sophie Lanzkron1, Alison R Moliterno, Edward J Norris
1Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. slanzkr@jhmi.edu
Background:
Acute chest syndrome (ACS) is a complication of sickle cell disease that can cause significant morbidity. Transfusion therapy has been shown to significantly increase oxygenation in patients with ACS and RBC exchange is considered the standard of care in patients at high risk of respiratory failure.
Case Report:
A patient with ACS and several high-risk features, including thrombocytopenia, profound anemia, bilateral pulmonary infiltrates, staphylococcal sepsis, and pulmonary embolism is presented. The patient refused transfusion on religious grounds and received 12 units of human polymerized Hb solution (poly SFH-P injection, PolyHeme, Northfield Laboratories) over the course of 13 days. The patient's respiratory status improved and she was discharged home without receiving RBC transfusions.
Conclusion:
This is the first reported case that describes the use of PolyHeme in a patient with sickle cell disease, ACS, and sepsis. This therapy is thought to have been lifesaving for this patient.