Transfusion associated graft-versus-host disease of children: a case report and literature review

Abstract

Objective To explore the clinical characteristics of transfusion associated graft-versus-host disease (TA-GVHD) of children. Methods On July 31, 2017, a 2-month-old male infant with TA-GVHD who was admitted to Shanghai Children′s Medical Center was selected as research subject into this study.Retrospective analysis was made on the clinical case data of the child and the clinical characteristics were summarized. " Transfusion associated graft-versus host disease" in Chinese and English respectively were used as key words in order to retrieve literature from China National Knowledge Infrastructure Database and Wanfang Database, as well as PubMed Database from establishment of database to December 2017. The characteristics, diagnosis, treatment and prognosis of children with TA-GVHD were summarized by reviewing related literature. The procedures followed in this study were in accordance with the requirements of the World Medical Association Declaration of Helsinki revised in 2013. Results ① This infant was a 2-month-old boy. Because of severe anemia due to infection, he received a non-irradiated erythrocyte transfusion from an unrelated donor before diagnosis of severe combined immunodeficiency. After the last blood transfusion, he presented with the clinical manifestations of fever, rash, diarrhea, elevated direct bilirubin, and hypoproliferative pancytopenia. The results of short tandem repeats (STR)-PCR showed that the genotypes of STR in oral epithelial tissue and blood samples were different, which suggested that there was a donor-derived cell implantation in the blood of the infant, and he was diagnosed as TA-GVHD. Through the results of immunodeficiency-related genes detection, the infant was finally diagnosed as severe combined immunodeficiency disease with TA-GVHD caused by X-linked IL2RG gene deficiency. ② Despite the active anti-infective and immunosuppressive therapy, the TA-GVHD infant died of sepsis and multiple organ failure 31 days after transfusion finally. ③ Literature review results indicated that the main underlying diseases of TA-GVHD in children were neonatal diseases and severe combined immunodeficiency diseases, presenting with fever, rash, elevated direct bilirubin, and hypoproliferative pancytopenia. Conclusions TA-GVHD is a rare, usual fatal, complication after receipt of any cellular blood component with viable lymphocytes. The clinical manifestations of TA-GVHD are lack of specificity, and the symptoms are easily concealed by primary diseases. If the clinicians does not know enough about this disease, it is prone to missed diagnosis. Therefore, clinicians should pay more attention to this disease. Key words: Blood transfusion; Graft vs host disease; Infant, newborn; Child; Severe combined immunedeficiency; γirradiation