Ղ Thalassemia is a very rare disease in Korea. Only14 mutations have been reported in South Korea to date. This is the first case of Ղ thalassemia intermedia due to hemoglobin Cagliari in Korea. The locus of this missense mutation is exon 3 of Ղ globin (Ղ60 Val→Glu). This is considered a de novo mutation of dominantly inherited Ղ 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 Ղ-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. (Korean J Hematol 2009;44:153-156.)

Keywords: Thalassemia, Parvovirus B19, Immunoglobulins, Intravenous