Intravenous gammaglobulin as rescue therapy in a patient with sickle cell and septic shock.

Introduction: We present a case involving a patient with sickle cell and hyposplenism, in which refractory septic shock quickly responded after the infusion of intravenous gammaglobulin (IV-GG) given as an adjuvant-rescue therapy

Case description: A 30-year-old African-American female with history of Sickle Cell disease was admitted for acute chest syndrome, septic shock and respiratory failure. Despite aggressive therapy the patient remained on two vasopressors and with persistent bacteremia. Within one day of starting IV-GG, both vasopressors (norepinephrine and vasopressin) were able to be discontinued.

Discussion: Patients with hyposplenism have functional opsonization failure. Infusion of IV-GG has been shown to improve such function in patients with hyposplenism. We were able to document a temporal association between IV-GG rescue therapy and septic shock improvement.

Conclusion: The utilization of intravenous gammaglobulin should be considered in patients with sickle cell disease and hyposplenism as an adjuvant therapy for refractory septic shock.