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KMID : 0366220090440030153
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Korean Journal of Hematology
2009 Volume.44 No. 3 p.153 ~ p.156
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A Case of ? Thalassemia Intermedia Due to Hemoglobin Cagliari (? 60 Val→Glu)
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Park Eun-Sil
Han Hye-Young
Lim Jae-Young
Park Sung-Sup
Kim Sun-Young
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Abstract
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? Thalassemia is a very rare disease in Korea. Only14 mutations have been reported in South Korea to date. This is the first case of ? thalassemia intermedia due to hemoglobin Cagliari in Korea. The locus of this missense mutation is exon 3 of ? globin (?60 Val→Glu). This is considered a de novo mutation of dominantly inherited ? thalassemia in patients previously having no family history of this conditions. The patient showed microcytic hypochromic anemia, jaundice, and splenomegaly, which were typical characteristics of ?-thalassemia intermedia. During follow-up, there was an episode of aplastic crisis due to Parvovirus B19 infection treated with intravenous immunoglobulin. We report this case with the related literature.
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KEYWORD
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Thalassemia, Parvovirus B19, Immunoglobulins, Intravenous
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