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Hyperphenylalaninemia in Malignant Histiocytosis and Virus‐Associated Hemophagocytic Syndrome
Author(s) -
Imashuku S.,
Kodo N.,
Esumi N.,
Morioka Y.,
Todo S.,
Furukawa N.,
Kinueasa A.
Publication year - 1988
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1988.tb10675.x
Subject(s) - medicine , hyperphenylalaninemia , malignant histiocytosis , tyrosine , histiocytosis , immunology , phenylalanine , histiocyte , gastroenterology , disease , amino acid , biology , biochemistry
It has been known for more than 2 decades that amino acid changes accompany various forms of infectious diseases. In some of these infections, an elevation in the phenylalanine/tyrosine ratio has been reported (1–4). Similar amino acid changes have also been described in protein calorie malnutrition (5) and chronic renal insufficiency (6). So far, however, such changes have not been clearly described in patients with hematologic neoplasias, especially for proliferative disorders of mononuclear macrophages. Nor has the actual occurrence of hyperphenylalaninemia in association with infectious or neoplastic disorders been reported. Recently, we encountered 2 patients with malignant histiocytosis (MH) (7) and a case of virus‐associated hemophagocytic syndrome (VAHS) (8). In all three patients the disease took a very rapid and fatal course with multiple organ dysfunction associated with hyperphenylalaninemia (>400 μmol/l) and an increased serum phenylalanine/tyrosine (Phe/Tyr) ratio (>4.00) (Table 1).