title: Secondary haemophagocytic lymphohistiocytosis triggered by postnatally acquired cytomegalovirus infection in a late preterm infant.
abstract: Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory condition with impairment of cytotoxic T-cells and natural killer cells. Causes in infants are mostly hereditary immune defects as well as various infectious triggering factors, amongst these cytomegalovirus (CMV). Vertical CMV transmission may occur in utero, during birth, and by breast feeding. Usually, a CMV infection transmitted via breast milk is symptomatic only in very immature preterm infants. We report on a late preterm infant born after 35 + 5 weeks of gestation with a birth weight of 1840 g, being admitted to our intensive care unit at the age of 9 weeks with acute enteritis and severe dehydration. After a prolonged recovery, the infant developed a sepsis-like condition with hyperpyrexia, hepatosplenomegaly, and pancytopenia. Combination with high ferritin levels (2809 μg/l), hypertriglyceridaemia (481 mg/dl), elevated soluble IL-2 receptor (sCD25, 9120 U/ml), and reduced perforin expression allowed diagnosis of HLH, caused by an acute CMV infection. Since connatal CMV infection had been ruled out earlier, we report the rare case of secondary HLH triggered by a postnatally acquired symptomatic CMV infection in an immunocompetent infant, most likely transmitted via breast milk. The infant was successfully treated with ganciclovir without need for immunosuppressive therapy.
authors.affiliation: Children's Hospital, University Hospital Wuerzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany. Silwedel_C@ukw.de.
authors.forename: Christine
authors.initials: C
authors.lastname: Silwedel
authors.affiliation: Children's Hospital, University Hospital Wuerzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany.
authors.forename: Eric
authors.initials: E
authors.lastname: Frieauff
authors.affiliation: Department of Paediatrics, Mutterhaus der Borromaeerinnen, Trier, Germany.
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Thomas
authors.affiliation: Children's Hospital, University Hospital Wuerzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany.
authors.forename: Johannes G
authors.initials: JG
authors.lastname: Liese
authors.affiliation: Children's Hospital, University Hospital Wuerzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany.
authors.forename: Christian P
authors.initials: CP
authors.lastname: Speer
mesh_terms: D000998:Antiviral Agents / Q000627:therapeutic use; D003587:Cytomegalovirus / Q000502:physiology*; D003586:Cytomegalovirus Infections / Q000150:complications* / Q000188:drug therapy / Q000635:transmission; D015774:Ganciclovir / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D008895:Milk, Human / Q000821:virology; D016896:Treatment Outcome
chemical_list: D000998:Antiviral Agents; D015774:Ganciclovir
keywords: Cytomegalovirus; Ganciclovir; Haemophagocytic lymphohistiocytosis; Preterm infant