Uracil-uric Refractory Anemia with Peroxidase Negative Neutrophils

Arakawa, Tsuneo; Wada, Yoshiro; Hayashi, Tadashi; Kakizaki, Rokuro; Chida, Noboru; Chiba, Ryuichi; Konno, Tasuke

doi:10.1620/tjem.87.52

Cited by 13 publications

(8 citation statements)

References 1 publication

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“…Our data suggest that MPO may be the major determinant of candidacidal activity in the human neutrophil. Occasional reports have associated leukocyte peroxidase deficiencies with other disorders (47)(48)(49)(50). With time it should be possible to evaluate the relative significance of hereditary _MPO deficiency as a predisposing cause of systemic candidiasis or other infections in man.…”

Section: Functional Studiesmentioning

confidence: 99%

Leukocyte myeloperoxidase deficiency and disseminated candidiasis: the role of myeloperoxidase in resistance to Candida infection

Lehrer¹,

Cline²

1969

J. Clin. Invest.

666

278

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A B S T R A C T The neutrophils and monocytes of a patient with disseminated candidiasis were found to lack detectable levels of the lysosomal enzyme myeloperoxidase (MPO), although they had normal levels of other granule-associated enzymes. Leukocytes from one of the patient's sisters also lacked detectable MPO; leukocytes from his four sons contained approximately one-third of mean normal peroxidase levels. Neither the patient nor his affected relatives had experienced frequent or unusual bacterial infections.The phagocytic activity of the patient's MPO-deficient neutrophils was intact, and the cells displayed normal morphologic and metabolic responses to phagocytosis. In contrast to normal leukocytes which killed 30.5 ±7.3% of ingested Candida albicans in 1 hr, however, the patient's neutrophils killed virtually none. His leukocytes also failed to kill the strain of C. albicans recovered from his lesions, as well as other Candida. species. These MPO-deficient neutrophils killed Serratia marcescens and Staphylococcus aureus 502A at an abnormally slow rate, requiring 3-4 hr to achieve the bactericidal effect attained by normal leukocytes after 45 min. No other abnormalities in his cellular or humoral immune responses were demonstrated.These findings suggest that hereditary MPO deficiency is transmitted as an autosomal recessive characteristic, that the homozygous state conveys enhanced susceptibility to disseminated candidiasis, and that MPO is necessary for candidacidal activity in human neutrophils.This work was presented in part at the National Meeting of the American Federation for Clinical Research, Atlantic City, N. J., and published in abstract form (1).

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Section: Functional Studiesmentioning

confidence: 99%

Leukocyte myeloperoxidase deficiency and disseminated candidiasis: the role of myeloperoxidase in resistance to Candida infection

Lehrer¹,

Cline²

1969

J. Clin. Invest.

666

278

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“…24 The absence of urinary excretion of orotic acid or of uracil excluded megalo blastic anemia due to orotic aciduria25 or uraciluria. 26 Vitamin B6 deficiency anemia was also unlikely, because abnormal tryptophan metabolites were not found in urine of our patient following an oral dose of tryptophan.…”

Section: Laboratory Findingsmentioning

confidence: 66%

Megaloblastic Anemia and Mental Retardation Associated with Hyperfolic-acidemia: Probably due to N<sup>5</sup> Methyltetrahydrofolate Transferase Deficiency

Arakawa

Narisawa

Tanno

et al. 1967

Tohoku J. Exp. Med.

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A probably new entity of metabolic error of folic acid was described of an infant whose clinical and biochemical characteristics were: 1) mental retarda tion and a marked dilatation of cerebral ventricles, 2) abnormally high serum L. casei folate activity, 3) abnormally high levels of folate precursor in erythrocytes, and 4) a marked rise in reticulocyte count by an exogenous folate supplementation.The results of investigation on tetrahydrofolate -dependent enzymes of liver specimens revealed a decreased activity of N5 methyltetrahydrofolate transferase of our own patient.It seems therefore likely that an impaired utilization of N5 methyltetrahy drofolate and its precursor in tissues due to a decreased activity of N5 methyl tetrahydrofolate transferase led to an abnormal accumulation of N5 methyl tetrahydrofolate and its precursor in serum and erythrocytes, and that trapping of folate compounds at an N5 methyltetrahydrofolate level caused a functional deficiency of folates with sequence of development of a megaloblastic change in the bone marrow and of an impaired purine biosynthesis of the brain with a marked dilatation of cerebral ventricles.

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“…Sato Peroxidase Negative Neutrophils" (Arakawa, 1965). In this case these neutrophils were also oxidase-negative.…”

mentioning

confidence: 60%

Peroxidase-negative Neutrophils in Arakawa-Higashi's Syndrome, in Encephalitis Economo, Further in “Healthy Brethren”

Sato

1965

Tohoku J. Exp. Med.

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Uracil-uric Refractory Anemia with Peroxidase Negative Neutrophils

Cited by 13 publications

References 1 publication

Leukocyte myeloperoxidase deficiency and disseminated candidiasis: the role of myeloperoxidase in resistance to Candida infection

Leukocyte myeloperoxidase deficiency and disseminated candidiasis: the role of myeloperoxidase in resistance to Candida infection

Megaloblastic Anemia and Mental Retardation Associated with Hyperfolic-acidemia: Probably due to N<sup>5</sup> Methyltetrahydrofolate Transferase Deficiency

Peroxidase-negative Neutrophils in Arakawa-Higashi's Syndrome, in Encephalitis Economo, Further in “Healthy Brethren”

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