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

M. Wakita, T. Matsui, M. Tsuzuki, T. Nomura, H. Miyazaki, H. Kojima, F. Maruyama, M. Okamoto, T. Ino, K. Ezaki, T. Sugihara, A. Kanzaki, Y. Yawata, M. Hirano

研究成果: Article査読

抄録

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.

本文言語English
ページ(範囲)265-270
ページ数6
ジャーナル[Rinshō ketsueki] The Japanese journal of clinical hematology
37
3
出版ステータスPublished - 03-1996

All Science Journal Classification (ASJC) codes

  • 医学(全般)

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