Titre du document / Document title

Idiopathic hyperammonemia : a frequency lethal complication of bone marrow transplantation

Auteur(s) / Author(s)

DAVIES S. M. ⁽¹⁾ ; SZABO E. ⁽¹⁾ ; WAGNER J. E. ⁽¹⁾ ; RAMSAY N. K. C. ⁽¹⁾ ; WEISDORF D. J. ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

⁽¹⁾ Department of Pediatrics, University of Minnesota, Minneapolis, MN, ETATS-UNIS

Résumé / Abstract

Idiopathic hyperammonemia (IHA) has been described as a rare complication of intensive chemotherapy, but there is little data regarding its occurrence after bone marrow transplantation (BMT). IHA is defined as elevated plasma ammonia concentrations (>200 μmol/l) in the absence of significant liver function abnormality. From a 21 year BMT database of 2358 patients, we have identified 12 patients (0.5%) with IHA, ages 19 to 46 years. Diagnoses included ALL (n = 2), AML (n = 4), CLL (n = 1), CML (n = 3) and aplastic anemia (n = 2). Eight received marrow from a matched sibling donor, three from an unrelated donor and one autologous marrow. IHA occurred between 14 and 106 days after transplant (median, 25 days). Most frequently patients presented with symptoms of a metabolic encephalopathy, with lethargy and confusion evolving into unresponsiveness, metabolic coma and in eight cases, seizures. At diagnosis of IHA, liver functions were normal or only modestly abnormal. Ten of the 12 patients died 1 to 9 days (median 3.5 days) after diagnosis of IHA despite treatment with combinations of dialysis and ammoniatrapping therapy. While IHA is a rare complication of BMT, it is associated with a high mortality. Early recognition of the syndrome by measurement of plasma ammonia concentrations in patients with neurological symptoms may improve outcome.

Revue / Journal Title

Bone marrow transplantation   ISSN 0268-3369   CODEN BMTRE9 

Source / Source

1996, vol. 17, n^o6, pp. 1119-1126 [7 page(s) (article)] (16 ref.)

Langue / Language

Anglais

Editeur / Publisher

Nature Publishing Group , Basingstoke, ROYAUME-UNI  (1986) (Revue)

Mots-clés anglais / English Keywords

Acute lymphocytic leukemia ; Acute myelocytic leukemia ; Chronic myelocytic leukemia ; Aplastic anemia ; Autograft ; Graft ; Homograft ; Bone marrow ; Hyperammonemia ; Idiopathic ; Treatment ; Encephalopathy ; Complication ; Human ; Acute ; Chronic ; Ammonia ; Hemopathy ; Lymphoproliferative syndrome ; Malignant hemopathy ; Myeloproliferative syndrome ; Transfusion ; Metabolic disorder ; Nervous system diseases ; Central nervous system disease ; Cerebral disorder ;

Mots-clés français / French Keywords

Leucémie lymphoblastique ; Leucémie myéloblastique ; Leucémie myéloïde ; Anémie aplasique ; Autogreffe ; Greffe ; Homogreffe ; Moelle osseuse ; Hyperammoniémie ; Idiopathique ; Traitement ; Encéphalopathie ; Complication ; Homme ; Aigu ; Chronique ; Ammoniac ; Hémopathie ; Lymphoprolifératif syndrome ; Hémopathie maligne ; Myéloprolifératif syndrome ; Transfusion ; Trouble métabolisme ; Système nerveux pathologie ; Système nerveux central pathologie ; Encéphale pathologie ;

Mots-clés espagnols / Spanish Keywords

Leucemia linfoblástica ; Leucemia mieloblástica ; Leucemia mieloidea ; Anemia aplástica ; Autoinjerto ; Injerto ; Homoinjerto ; Médula ósea ; Hiperamonemia ; Idiopático ; Tratamiento ; Encefalopatía ; Complicación ; Hombre ; Agudo ; Crónico ; Amoníaco ; Hemopatía ; Linfoproliferativo síndrome ; Hemopatía maligna ; Mieloproliferativo síndrome ; Transfusión ; Trastorno metabolismo ; Sistema nervioso patología ; Sistema nervosio central patología ; Encéfalo patología ;

Localisation / Location

INIST-CNRS, Cote INIST : 21176, 35400006007406.0340