title: Early evidence of bone marrow dysfunction in children with indeterminate fulminant hepatic failure who ultimately develop aplastic anemia.
abstract: In children, aplastic anemia (AA) is a common complication associated with fulminant hepatic failure (FHF). The objective of this study was to determine whether specific pretransplantation clinical and laboratory characteristics can be used to distinguish between patients with FHF who are at higher risk of developing AA. We performed a retrospective case-control study to evaluate the clinical and laboratory characteristics of those patients who presented with evidence of FHF and eventually developed aplastic anemia. We identified nine patients with AA, and all had the indeterminate form of FHF and underwent liver transplantation (LTx). The AA patients were compared with a control group of 47 patients with indeterminate FHF that underwent transplantation and did not develop AA. We found that males were over-represented in the group of patients that developed AA (p = 0.01). Furthermore, during the pretransplant period, the AA group had a significantly lower white count (p = 0.005), absolute lymphocyte count (p = 0.004), and platelet count (p = 0.019) when compared with controls. We conclude that evidence of early bone marrow dysfunction is apparent before liver transplantation and the development of AA in a subset of patients with the indeterminate form of FHF.
authors.affiliation: Divisions of Gastroenterology, Department of Pediatrics, Mattel Children's Hospital, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
authors.forename: Ricardo A
authors.initials: RA
authors.lastname: Molina
authors.forename: Lirona
authors.initials: L
authors.lastname: Katzir
authors.forename: Chris
authors.initials: C
authors.lastname: Rhee
authors.forename: Leslie
authors.initials: L
authors.lastname: Ingram-Drake
authors.forename: Ted
authors.initials: T
authors.lastname: Moore
authors.forename: Paul
authors.initials: P
authors.lastname: Krogstad
authors.forename: Martín G
authors.initials: MG
authors.lastname: Martín
mesh_terms: D000293:Adolescent; D000741:Anemia, Aplastic / Q000378:metabolism*; D015415:Biomarkers / Q000097:blood; D001792:Blood Platelets / Q000378:metabolism; D001853:Bone Marrow / Q000473:pathology*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007962:Leukocytes / Q000378:metabolism; D017114:Liver Failure, Acute / Q000378:metabolism*; D008297:Male
chemical_list: D015415:Biomarkers