Severe bacteria-associated hemophagocytic lymphohistiocytosis in an extremely premature infant.
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.
Edner
Josefine
J
Childhood Cancer Research Unit, Department of Women and Child Health, Karolinska University Hospital, Stockholm, Sweden.
Rudd
Eva
E
Zheng
Chengyun
C
Dahlander
Andreas
A
Eksborg
Staffan
S
Schneider
E Marion
EM
Edner
Ann
A
Henter
Jan-Inge
JI
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
D000305:Adrenal Cortex Hormones; D003603:Cytotoxins; D007136:Immunoglobulins; D005047:Etoposide; D003907:Dexamethasone