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Article in Japanese

Neonatal hemophagocytic lymphohistiocytosis caused by coxsackievirus B4 with different clinical courses in monochorionic diamniotic twins

Saho SHIMA1) , Yuhei TANAKA1) , Mariko TERAMACHI1) , Masahiro KINOSHITA1) , Kenji GOTOH1,2)

Hemophagocytic lymphohistiocytosis (HLH) in the neonatal period is an extremely rare disease. Herein we report the cases of monochorionic diamniotic twin with HLH caused by enterovirus. The infants were born by emergency cesarean section at 32 weeks gestation due to maternal pneumonia and heart failure. Case1 had apnea, purpura, and edema on the third day. Bone marrow examination on the seventh day revealed hemophagocytosis and Case1’s illness was diagnosed as HLH. She was treated intensively with immunoglobulin, steroids, and anticancer agents, but developed myocarditis and died on 29 days of age. Case2 was administrated an artificial lung surfactant immediately after birth due to neonatal respiratory distress syndrome. Case2 showed apnea and thrombocytopenia on the sixth day. Considering the history of case 1, she was administered to intravenous immunoglobulin and steroids for the treat of HLH. On the seventh day, Coxsackievirus B4 (CoxB4) was detected from the blood and stool of both infants by polymerase chain reaction, and the HLH in both infants was thought to be caused by CoxB4. The two cases in this study were thought to be severe because the mothers were not immune to CoxB4. If the mother presents with viral pneumonia just before delivery, careful observation and care of the infants are necessary in consideration of HLH.

1) Department of Pediatrics and Child Health, Kurume University School of Medicine
2) Department of Infection Control and Prevention, Kurume University School of Medicine

Key words hemophagocytic lymphohistiocytosis, Coxsackievirus B4, neonate, twins, myocarditis
Received November 20, 2021
Accepted February 15, 2021

34 (1):9─16,2022