Failure of microchromatographic measurement of fetal hemoglobin in β0 thalassemia - hereditary persistence of fetal hemoglobin

J. S. Krauss, M. H. Jonah, Lawrence D Devoe, C. G. Pantazis

Research output: Contribution to journalArticle

3 Scopus citations

Abstract

We report microchromatographic measurement of fetal hemoglobin (HbF) proportions in a 36-year-old African-American multigravida woman. At 34 weeks she delivered a 630-g male infant who subsequently did well. Hemoglobin electrophoresis of the hemolysate revealed nearly 100% HbF without HbA, an extremely unusual naturally occurring sample. Family studies revealed a combination of hereditary persistence of fetal hemoglobin (HPFH) and β0 thalassemia minor. Southern blot technique confirmed heterozygous α2 thalassemia and HPFH but failed to identify the β thalassemic lesion. The absence of HbA and the very high amounts of HbF led us to measure HbF by several methods to confirm the accuracy of microchromatography of HbF at values approaching 100%. HPLC revealed a 14% F1 suggestive of microchromatographic underestimation due to glycated HbF. We conclude that cation-exchange microchromatography and the Betke method of alkali denaturation underestimate HbF values as they approach 100% and do not recommend these procedures in this rare situation.

Original languageEnglish (US)
Pages (from-to)2325-2327
Number of pages3
JournalClinical Chemistry
Volume38
Issue number11
StatePublished - Dec 1 1992

ASJC Scopus subject areas

  • Clinical Biochemistry

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