溶血性貧血を巡る最近の話題

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  • Recent Topics in Hemolytic Anemias.

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Most pediatric hematologists are indifferent to hemolytic anemias. In this article, several interesting researches mainly by Idiopathic Disorders of Hematopoietic Organs Research Committee, the Ministry of Health and Welfare of Japan are presented. Hereditary spherocytosis and elliptocytosis are consequences of a deficiency or a mutation of membrane skeletal proteins such as spectrin and ankyrin. Congenital dyserythropoietic anemia (CDA) type II (HEMPAS) is the most common CDA. Band 3 glycoproteins from HEMPAS red cells revealed truncated hybrid type structures. This suggests that the primary enzyme deficiency of HEMPAS is in N-acetylglucosaminyltransferase II (GnTII) or α-mannosidase II (α-MII). The incidence of hemolytic uremic syndrome (HUS) has an increasing tendency. Verotoxin-producing Escherichia coli has been recognized as the gastrointestinal pathogen most frequently associated with HUS. Affected erythrocytes of patients with paroxysmal nocturnal hemoglobinuria (PNH) are deficient in the complement regulatory proteins : GPI anchored proteins. cDNA cloning of deficient enzyme is in progress. High-dose intravenous gammaglobulin therapy for neonatal immune hemolytic anemia caused by blood group incompatibility seems to be useful.

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