The Turkish Journal of Pediatrics 2021 , Vol 63 , Num 6
Covid-19 in a patient with Familial Hemophagocytic Lymphohistiocytosis in children
V. Hülya Üzel 1 ,Kamil Yılmaz 2
1 Division of Pediatric Hematology, Dicle University Faculty of Medicine, Diyarbakır, Turkey
2 Department of Pediatrics, Dicle University Faculty of Medicine, Diyarbakır, Turkey
DOI : 10.24953/turkjped.2021.06.021 Background. Based on the information obtained so far, COVID- 19 is relatively mild in children. We will present a 6-month-old male patient infected with COVID -19 in April 2020, while receiving HLH 2004 chemotherapy protocol with the diagnosis of familial (Genetic / Primary) Hemophagocytic Lymphohistiocytosis (HLH).

Case. Herein we present a case accompanied by a defective perforin gene defect in the primary HLH pathogenesis, Covid-19 infection with the presence of fever and hyperferritinemia, which was evaluated in favor of reactivation and the patient was given both the HLH-2004 chemotherapy protocol treatment and COVID -19 therapy as recommended by the guidelines. Our patient improved clinically and in terms of laboratory test results at the end of the 15th day of hospitalization and was discharged.

Conclusions. It should be remembered that COVID-19 can be seen with different clinical manifestations in the pediatric age group, and COVID-19 tests should be recommended, especially in children with immunosuppression and fever. Keywords : COVID-19, hemophagocytic lymphohistiocytosis, perforin

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