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Hyperammonemia: elevated ammonia levels in multiple myeloma

2004; Elsevier BV; Volume: 116; Issue: 3 Linguagem: Inglês

10.1016/j.amjmed.2003.06.010

1555-7162

Joseph R. Holahan,

Epilepsy research and treatment

Ammonia (NH3) is a surrogate marker for the numerous amines that are elevated in hepatic failure. It or other amines are responsible for the mental changes that occur in this context. The most common causes of hyperammonemia are congenital disorders of urea metabolism and severe dysfunction of the liver. This is a report of a patient with multiple myeloma whose hyperammonemic encephalopathy derived from the myeloma rather than liver dysfunction. An 82-year-old female Hispanic patient had several months of low back and abdominal pain. An evaluation revealed cholecystitis, and a cholecystectomy was performed. The pain, however, was not relieved, and the patient developed postoperative complications including renal failure and encephalopathy. She was found to have an extremely elevated serum ammonia level of 119 μ/dL (reference: 100 μ/dL), has recently been brought to our attention. In several cases, in vitro studies have verified excess production of NH3 by myeloma cells in culture (6Matsuzaki H. Matsuno F. Yoshida M. et al.Coma, hyperviscosity syndrome, hyperammonemia and myelofibrosis in a patient with IgG, lambda type multiple myeloma.Rinsho Ketsueki. 1989; 30: 361-365PubMed Google Scholar, 7Matsuzaki H. Matsuno F. Yoshida M. et al.Human myeloma cell line (KHM-4) established from a patient with multiple myeloma associated with hyperammonemia.Intern Med. 1992; 31: 339-343Crossref PubMed Scopus (17) Google Scholar, 8Kozuka T. Fukada S. Imai T. Sekaki T. Clinical significance of a multiple myeloma cell line derived from a case associated with hyperammonaemia.Rinsho Ketsueki. 2000; 41: 1220-1225PubMed Google Scholar). The patient in this report, as well as several others, failed to respond to standard treatments for hyperammonemia, but did respond to chemotherapy with normalization of NH3 levels and by regaining consciousness (2Matsuzaki H. Uchiba M. Yoshimura K. et al.Hyperammonaemia in multiple myeloma.Acta Hematol. 1990; 84: 130-134Crossref PubMed Scopus (30) Google Scholar, 4Martinelli G. Peccatori F. Ullrich B. et al.Clinical manifestation of severe hyperammonaemia in patients with multiple myeloma.Ann Oncol. 1997; 8: 811Crossref PubMed Scopus (11) Google Scholar). Multiple myeloma should be included in the differential diagnosis of hyperammonemia. This case and the other reported cases have usually had advanced disease.