皮膚光線過敏症と赤血球プロトポルフィリンの増加を認めた鉄芽球性貧血の1例

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タイトル別名
  • A Case of Sideroblastic Anemia with Dermal Photosensitivity and Increased Erythrocyte Protoporphyrin
  • ヒフ コウセン カビンショウ ト セッケッキュウ プロトポルフィリン ノ ゾウ

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抄録

A 57-year-old male had noted dermal photosensitivity and shortness of breath 1 year before admission. Since then he had been treated with iron or vitamin B6 in other hospitals but the symptoms did not improve. In July 1980, he was admitted to our hospital for precise evaluation. Physical examinations revealed slight anemia and atrophy of the skin at the area exposed to sun light. Peripheral blood examinations showed microcytic hypochromic anemia with marked poikilocytosis and dimorphism. Bone marrow smears showed erythroid hyperplasia with increase in the number of ringed sideroblasts. From these findings, he was diagnosed as having acquired sideroblastic anemia. Since protoporphyrin in red blood cells increased markedly, dermal photosensitivity was caused by similar mechanisms as erythropoietic protoporphyria. Moreover, chromosomal abnormalities of the marrow cells were noted. Granulocyte-macrophage colony formation and erythroid colony formation were markedly suppressed. From these findings, it can be considered that both sideroblastic anemia and increased levels of protoporphyrin in red blood cells occurred secondary to mutation of the hemopoietic stem cells.

収録刊行物

  • 臨床血液

    臨床血液 22 (12), 1971-1976, 1981

    一般社団法人 日本血液学会

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