赤血球膜脂質，とくにホスファチジルコリンの高値とナトリウム輸送能の亢進を示した先天性非球状赤血球性溶血性貧血の1症例

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タイトル別名
  • A Case of Congenital Hemolytic Anemia Associated with High Red Cell Membrane Phosphatidyl Choline and Enhanced Na<sup>+</sup>-Transport
  • セッケッキュウ マク シシツ トクニ ホスファチジルコリン ノ タカネ ト ナ

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A 18-year-old Japonese girl with congenital hemolytic anemia associated with elevated red cell membrane phosphatidyl choline and enhanced Na+-transport is described. At the age of 4, she was diagnosed as having congenital hemolytic anemia after detailed examination. She was hospitalized twice thereafter because of persistent jaundice and repeated abdominal pain before undergoing splenectomy and cholecystotomy at the age of 14 with a tentative diagnosis of hereditary spherocytosis. Her hemolytic anemia however did not improve appreciably. Because of recurrent cholelithiasis, the cholecystectomy was performed at 18 years of age. Soon thereafter she was referred to us for further investigation. Her Hb was 11.3 g/dl, reticulocytes 29.3% and WBC 7,700/ul. A moderate macrocytosis (MCV 108μ8) with target cells was noted. Her bilirubin was 10.5 mg/dl with 8.3 mg/dl indirect-reacting bilirubin The osmotic fragility was decreased and autohemolysis showed Dacie's type I pattern. Hemoglobin analysis and red cell enzyme activities were unremarkable. Her red cell membrane showed increased cholesterol and phospholipids per RBC along with elevated phosphatidyl choline (33.0%) and depressed phosphatidyl ethanolamine (25.3%). Sodium transport was markedly elevated. Her parents and two siblings were all normal with respect to the red cell lipids and sodium transport. There were no similar diseases in her family tree. Thus, her hemolytic anemia appeared to be related to the abnormal red cell lipid constitution, analogous to that reported by Jaffé et al as high phosphatidyl choline hemolytic anemia.

収録刊行物

  • 臨床血液

    臨床血液 23 (11), 1816-1822, 1982

    一般社団法人 日本血液学会

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