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KMID : 0384119930130020225
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Korean Journal of Clinical Pathology
1993 Volume.13 No. 2 p.225 ~ p.232
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Analysis of Routine Test Results for the Diagnosis of Paroxysmal Nocturnal Hemoglobinuria
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김선희
박성섭/유종현/조한익/김병국
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Abstract
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Paroxysmal nocturnal hemoglobinuria(PNH) is now accepted as a disease in which bone marrow derived cells are deficient in phosphatidylinositolglycan(PIG)-anchored surface molecules and new diagnostic methods detecting them have recently been
described.
But Ham's test is still used as a choice for the diagnosis of PNH. In the present study, we analysed usefulness of conventional diagnostic methods of PNH. We retrospectively reviewed laboratory data of 40 patients with positive Ham's test at
Seoul
national University Hospital from 1984 to 1992. Among the 40 patients with positive Ham's test, four were negative in sucrose lysis test and repeated Ham's tests were needed to get positive results in 7 cases. Decreased leukocyte alkaline
phosphatase
and hemosiderinuria were observed in 74% and 61% of the cases, respectively. In the marrow study, six cases showed aplastic marrow and 19 cases showed distinct dyserythropoietic features, which were suggeetive of the myelodysplastic syndromes.
Autoimmune hemolysis was detected in four cases. Ham's test should be repeated when PNH is highly suspicious clinically. None of the routine tests including ham's test and bone marrow study is confirmatory for the diagnosis of PNH. New tests
detecting
basic defects of PNH are considered to be necessary.
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