title: Haemophagocytic lymphohistiocytosis presenting as neonatal liver failure: A case series.
abstract: OBJECTIVE
  Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome with liver involvement varying from mild dysfunction to severe fulminant failure. The aim of this study was to present a case series of four HLH patients presenting with acute liver failure (ALF) in the neonatal period.
  
  
  METHODS
  All four patients were neonates at the onset of symptoms. They presented to Cairo University Pediatric Hospital with ALF; they underwent prompt investigations including determination of ferritin, fibrinogen, and triglyceride levels as part of our ALF workup. Further investigations were tailored according to the associated clinical features and the results of preliminary investigations.
  
  
  RESULTS
  HLH was diagnosed according to HLH-2004 criteria. Three patients fulfilled at least five out of eight criteria. Fever, splenomegaly, elevated ferritin levels, and low fibrinogen levels were present in all patients. The fourth patient had a serum ferritin level >10,000ng/ml, favouring the diagnosis of HLH, despite fulfilling only four out of eight criteria. For three patients, positive consanguinity and previous sibling death were reported, suggesting a genetic aetiology of HLH.
  
  
  CONCLUSIONS
  ALF can be the presenting feature of HLH; thus, a high index of suspicion is necessary. Fever is a hallmark, especially in neonates. Diagnosis is important for this potentially treatable condition.
authors.affiliation: Department of Pediatrics, Kasr Alainy Medical School, Cairo University, El Saray Street, El Manial, Cairo 11956, Egypt. Electronic address: halamohsen@kasralainy.edu.eg.
authors.forename: Hala
authors.initials: H
authors.lastname: Abdullatif
authors.affiliation: Department of Pediatrics, Kasr Alainy Medical School, Cairo University, El Saray Street, El Manial, Cairo 11956, Egypt. Electronic address: mohsennabil2000@yahoo.com.
authors.forename: Nabil
authors.initials: N
authors.lastname: Mohsen
authors.affiliation: Department of Pediatrics, Kasr Alainy Medical School, Cairo University, El Saray Street, El Manial, Cairo 11956, Egypt. Electronic address: rokaya_mohsen@kasralainy.edu.eg.
authors.forename: Rokaya
authors.initials: R
authors.lastname: El-Sayed
authors.affiliation: Department of Clinical and Chemical Pathology, Kasr Alainy Medical School, Cairo University, El Saray Street, El Manial, Cairo 11956, Egypt. Electronic address: fatma.elmougy@kasralainy.edu.eg.
authors.forename: Fatma
authors.initials: F
authors.lastname: El-Mougy
authors.affiliation: Department of Pediatrics, Kasr Alainy Medical School, Cairo University, El Saray Street, El Manial, Cairo 11956, Egypt. Electronic address: hanaakaraksy@kasralainy.edu.eg.
authors.forename: Hanaa
authors.initials: H
authors.lastname: El-Karaksy
mesh_terms: D003241:Consanguinity; D017809:Fatal Outcome; D005260:Female; D005293:Ferritins / Q000097:blood; D005334:Fever / Q000209:etiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D017114:Liver Failure, Acute / Q000209:etiology*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics; D008297:Male; D013163:Splenomegaly / Q000209:etiology
chemical_list: D005293:Ferritins
keywords: Acute liver failure; Ferritin; HLH; Haemophagocytic lymphohistiocytosis; Neonates