P0731 / #2140: SARS-COV-2-RELATED MULTISYSTEM INFLAMMATORY SYNDROME IN FAMILIAL REFRACTORY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: A CASE REPORT

Abstract

AIMS & OBJECTIVES: To describe how the clinical course of children positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) tested with reversetranscriptase polymerase chain reaction (RT-PCR) has changed lately from non-severe to life threatening cases. METHODS: Case report. A 2-month-old baby arrived at the emergency department for fever, anemia, leukopenia and thrombocytopenia, he was hospitalized and diagnosed with severe dengue. A week later, he started with respiratory symptoms and fever, he was positive for SARS-CoV-2 based on RT-PCR. Two weeks later, persistent fever, C-Reactive protein and D-dimer elevation, lymphocytopenia, anemia and thrombocytopenia integrated diagnosis of multisystem inflammatory syndrome (MIS-C) treated with intravenous immune globulin, systemic glucocorticoid and anticoagulation therapy in the intensive care unit. Nevertheless, he had persistent fever, hepatosplenomegaly, pancytopenia, hypertriglyceridemia and high serum IL-6 levels;bone marrow aspiration revealed an hemophagocytic lymphohistiocytosis (HLH), so treatment with steroid, cyclosporine and cytarabine was administrated. Antibiotic therapy was added because of clinical symptoms for septic shock. Multiple transfusions because of refractory anemia and thrombocytopenia, vasopressor management and mechanical ventilation were needed. However, because of his insidious clinical course and history of infection with SARS-CoV-2, management with inhibitor of JAK signalling was added. Physical examination revealed silver hair, suggestive for a familial HLH secondary to Griscelli syndrome type 2. RESULTS: In the end, multiple organ dysfunction (cardiovascular, hematologic, respiratory, renal, neurologic). CONCLUSIONS: MIS-C was secondary to an immunemediated injury triggered by viral infections with an overall mortality greater than 50% in previously healthy children, however additional HLH resulted in fatal outcome.