title: Severe bacteria-associated hemophagocytic lymphohistiocytosis in an extremely premature infant.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition with high mortality. We report an extremely premature girl, born in the 24th gestational week (BW 732 g), that during her second month developed a severe HLH subsequent to a Serratia marcescens septicemia, with hepatosplenomegaly, cytopenias, hyperbilirubinemia (mostly conjugated, total bilirubin 916 mumol/L), hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia (21266 mug/L), and elevated sIL-2 receptor levels. Genetic analysis revealed no PRF1, STX11 or UNC13D gene mutations. Treatment was provided according to the HLH-2004 protocol with etoposide, dexamethasone, and immunoglobulin, but no cyclosporin because of immature kidneys. She recovered fully from the HLH but developed a severe retinopathy as well as green teeth secondary to the hyperbilirubinemia. We conclude that secondary, bacteria-associated HLH can develop in premature infants, and that HLH can be treated with cytotoxic therapy also in premature infants. It is important to be aware of HLH in premature infants, since it is treatable.
authors: Childhood Cancer Research Unit, Department of Women and Child Health, Karolinska University Hospital, Stockholm, Sweden.
authors: Josefine
authors: J
authors: Edner
authors: Eva
authors: E
authors: Rudd
authors: Chengyun
authors: C
authors: Zheng
authors: Andreas
authors: A
authors: Dahlander
authors: Staffan
authors: S
authors: Eksborg
authors: E Marion
authors: EM
authors: Schneider
authors: Ann
authors: A
authors: Edner
authors: Jan-Inge
authors: JI
authors: Henter
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D003603:Cytotoxins / Q000008:administration & dosage / Q000627:therapeutic use; D003907:Dexamethasone / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D006932:Hyperbilirubinemia / Q000175:diagnosis; D007136:Immunoglobulins / Q000008:administration & dosage; D052577:Infant, Extremely Low Birth Weight; D007231:Infant, Newborn; D007234:Infant, Premature; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000188:drug therapy* / Q000382:microbiology; D012178:Retinopathy of Prematurity; D018805:Sepsis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy* / Q000382:microbiology; D016868:Serratia Infections / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D012706:Serratia marcescens
chemical_list: D000305:Adrenal Cortex Hormones; D003603:Cytotoxins; D007136:Immunoglobulins; D005047:Etoposide; D003907:Dexamethasone