Five cases of hereditary high red cell membrane phosphatidylcholine hemolytic anemia in three families

Five cases of hereditary high red cell membrane phosphatidylcholine hemolytic anemia in three families were described. All cases were clinically manifested by jaundice and splenomegaly. Hemolysis was evident from indirect hyperbilrubinemia, reticulocytosis and decrement of serum haptoglobin. Red blo...

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Published inRinshō ketsueki Vol. 37; no. 3; p. 265
Main Authors Wakita, M, Matsui, T, Tsuzuki, M, Nomura, T, Miyazaki, H, Kojima, H, Maruyama, F, Okamoto, M, Ino, T, Ezaki, K, Sugihara, T, Kanzaki, A, Yawata, Y, Hirano, M
Format Journal Article
LanguageJapanese
Published Japan 01.03.1996
Subjects
Adult
Anemia, Hemolytic, Congenital - blood
Anemia, Hemolytic, Congenital - genetics
Erythrocyte Membrane - metabolism
Family Health
Humans
Male
Middle Aged
Phosphatidylcholines - blood
Sodium - metabolism
Splenectomy
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Summary:Five cases of hereditary high red cell membrane phosphatidylcholine hemolytic anemia in three families were described. All cases were clinically manifested by jaundice and splenomegaly. Hemolysis was evident from indirect hyperbilrubinemia, reticulocytosis and decrement of serum haptoglobin. Red blood cells showed morphological abnormalities such as poikylocytosis, anisocytosis and target cells on blood smears. Both direct and indirect Coombs' tests were negative. Ham test, sugar water test and hemoglobin electrophoresis showed no abnormalities. Osmotic fragility test showed decreased membrane fragility. Lipid analysis of red cell membrane showed increment of phosphatidylcholine content and decrement of sphingomyelin content, although plasma lipids were essentially normal. Influx and efflux of sodium through the red cell membrane were both increased. Splenectomy was performed without effect on one patient and the mother of other patients.
ISSN:0485-1439
DOI:10.11406/rinketsu.37.265