Cytokine storm in HSCT for severe combined immunodeficiency infant with SARS-COV-2

Abstract

Hematopoietic stem cell transplant (HSCT) is the only potentially curative option for severe combined immunodeficiency (SCID) as they are extremely vulnerable to infections. Immunocompromised children are at a higher risk of SARS-CoV-2 infection with prolonged virus shedding, but have a milder disease unlike adults. However, mortality risk increases with neutropenia and in the early transplant period. For these reasons, HSCT is generally deferred when a patient is infected with SARS-COV-2. This decision has to be individualized taking into account the risk of disease progression with delay in transplant. We describe a case of a SCID infant, who had multiple, life-threatening infections (methicillin-resistant Staphylococcus aureus liver abscess, Escherichia coli sepsis, and disseminated Bacillus Calmette-Guerinosis) referred for HSCT. He unfortunately developed SARS-COV-2 infection after the conditioning was commenced for haploidentical stem cell transplant. Foreseeing many challenges with COVID, the transplant was undertaken in the pediatric intensive care unit (PICU) setting. Anticipation, recognition, and timely intervention in the PICU of exaggerated posttransplant cytokine release syndrome and pancreatitis enabled a successful outcome. To the best of our knowledge, this is the youngest pediatric HSCT performed to date with active SARS-COV-2 and first in India.