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發作性寒冷血色素尿症

PAROXYSMAL COLD HEMOGLOBINURIA

중앙의학 1961년 1권 2호 p.209 ~ 214
李聖洙, 金基賢, 李正基,
소속 상세정보
李聖洙 (  ) - 서울대학교 醫科大學
金基賢 (  ) - 서울대학교 醫科大學
李正基 (  ) - 서울대학교 醫科大學

Abstract


A case of clinically and clinical-pathologically proven paroxysmal cold hemoglobinuria in a 14 year old Korean male was described. This patient was diagnosed to have probable congenital syphilis on the ground that his blood was cardiolipin flocculation positive and Wassermann positive, and he had saddle nose,´ Hutchinson´s teeth and neuropathy, although his parents syphilis was not confirmed. The patient suffered from occasional hemoglobinuria accompanied by chills, fever, headache, abdominal pain and fatigability, after he was exposed to cold even in summer season.
His serum contained cold autohemolysins that were confirmed by Donath-Landsteiner test, etc. The patient had not cold agglutins in his blood, and fragility test of his red blood cells showed values within normal limits. After hemoglobinuric crisis, marked anemia, leukocytosis and increased number of reticulocytes in his peripheral blood were observed and the bone marrow revealed exaggerated erythropoiesis.

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