Hemophagocytic Lymphohistiocytosis in the Neonatal Period

Abstract

A report of hemophagocytic lymphohistiocytosis (HLH) in the neonatal period is rare. I was able to find 26 neonatal HLH cases from a medical magazine web in Japan and I analyzed them. The cause of the disease was not identified in more than half of the patients. However, herpes virus or enterovirus that was regarded as infection from the mother was identified in ten patients. No fever was recorded in premature infants. Thrombocytopenia, elevated AST, LDH and hyperferritinemia were useful markers for an early diagnosis of HLH. There were few cases that measured soluble interleukin-2 receptor or urine β2-microglobuline, which, however, rose in almost all cases. About half of the infants lived, but the survival for the eight patients that satisfied diagnostic criteria of HLH-2004 was only two. Improvement of the prognosis may be obtained by early diagnosis followed by chemo-immunotherapy such as HLH-2004. As it is very likely that a serious infectious disease may develop after chemo-immunotherapy, the prevention of such an infection is necessary and we must keep an eye on it.