Journal List > Korean J Hematol > v.40(1) > 1032651

Kim, Kim, Park, Park, Cheong, Lee, Min, Hahn, and Kie: A Case of Serum Amino Acid Disturbance with Hyperammonemia in Patient with Primary Amyloidosis

Abstract

There have been reports that hyperammonemia and amino acid disturbance can cause loss of consciousness in patients with multiple myelomas and normal liver function. We experienced a case of a 71-years-old female patient with amyloidosis, who had shown disturbance of consciousness. At that time, the serum ammonia level was elevated; serum amino acids disturbance was also noted. In particular, the decrease in branched-chain amino acids and increase in aromatic amino acids results in a low Fisher ratio. The Fisher ratio, the ratio of branched-chain to aromatic amino acids, has been suggested as an important indicator of consciousness disturbance. We report, for the first time in Korea, a case of amyloidosis, with mental disturbance due to serum amino acid disturbance.

REFERENCES

1). Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med. 1997; 337:898–909.
crossref
2). Lee JH, Ko YW, Maeng HY, et al. A case of serum amino acid disturbance with hyperammonemia in a patient with multiple myeloma. Korean J Hematol. 2002; 37:84–7.
3). Seo YM, Eom HS, Kim JK, et al. A case of hyperammonemic encephalopathy with serum amino acid alteration in multiple myeloma. Korean J Hematol. 2003; 38:64–7.
4). Matsuzaki H, Hata H, Sonoki T, et al. Serum amino acid disturbance in multiple myeloma with hyperammonemia. Int J Hematol. 2006. 131–7.
5). Matsuzaki H, Uchiba M, Yoshimura K, et al. Hyperammonemia in multiple myeloma. Acta Haematol. 1990; 84:130–4.
crossref
6). Fujii S, Fukuda S, Sezaki T, Murakami M. Clinicopathological study of multiple myeloma associated with hyperammonemia. Rinsho Ketsueki. 1998; 39:27–33.
7). Watson AJ, Chambers T, Karp JE, Risch VR, Walker WG, Brusilow SW. Transient idiopathic hyperammo-naemia in adults. Lancet. 1985; 2:1271–4.
crossref
8). Takimoto Y, Imanaka F, Hayashi Y, Morioka S. A patient with ammonia-producing multiple myeloma showing hyperammonemic encephalopathy. Leukemia. 1996; 10:918–9.
9). Kwan L, Wang C, Levitt L. Hyperammonemic encephalopathy in multiple myeloma. N Engl J Med. 2002; 346:1674–5.
crossref
10). Holahan JR. Hyperammonemia: elevated ammonia levels in multiple myeloma. Am J Med. 2004; 116:210–1.
crossref
11). Weng TI, Shih FF, Chen WJ. Unusual causes of hyperammonemia in the ED. Am J Emerg Med. 2004; 22:105–7.
crossref
12). Tsunoda S, Sasaki R, Miwa A, Sakurabayashi I, Miura Y. Coma, hyperviscosity syndrome, hyperammonemia and myelofibrosis in a patient with IgG, lambda type multiple myeloma. Rinsho Ketsueki. 1989; 30:361–5.
13). Caminal L, Castellanos E, Mateos V, Astudillo A, Moreno C, Dieguez MA. Hyperammonaemic encephalopathy as the presenting feature of IgD multiple myeloma. J Intern Med. 1993; 233:277–9.
crossref

Fig. 1.
Some arteriolar walls are thickened by amorphous material deposits. There are multifocal chronic inflammatory cell infiltration and fibrosis associated tubular atrophy. The deposits are Congo-red positive (A: H&E ×400, B: Congo-red, ×400).
kjh-40-54f1.tif
Fig. 2.
Brain computed tomography shows no abnormal findings.
kjh-40-54f2.tif
Table 1.
Characteristics of serum amino acid disturbance in this case (nmol/mL)
Compounds Results Results Normal range
Hospital day D+9 D+16  
Glycine 91.2 271.1 151.0~490.0
Tylosine 35.9 9.8 34.0~112.0
Valine 109.2 139.7 119.0~336.0
Leucine 53.5 76.4 72.0~201.0
Isoleucine 35.0 63.6 30.0~108.0
Phenyalanine 330.1 22.3 35.0~85.0
Fisher ratio 0.53 8.71 2.6~4.3

Matsuzaki, et al (1990) Fisher ratio=(Val+Ile+Leu)/(Phe+ Tyr).

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