臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
症例
骨髄低形成をともない,臨床経過中にPNH赤血球の消失と補体感受性の変化を観察し得たPNHの1症例
七島 勉吉田 幹男寺澤 崇今野 一郎三田 正行内田 立身刈米 重夫
著者情報
ジャーナル 認証あり

1987 年 28 巻 4 号 p. 564-569

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A 57-year-old male was admitted to our department because of palpitation, shortness of breath and pancytopenia on June 18, 1985. On admission, conjunctiva was anemic, and petechiae and ecchymoses were found in the skin. Laboratory findings revealed pancytopenia, a reticulocyte count of 24‰, positive Ham's test, NAP score of 68, red cell acetylcholine esterase of 0.8 U, hemosiderinuria, LDH 842 WrU, and haptoglobin less than 10mg/dl. Bone marrow showed hypocellularity (0.85×104/cmm). Diagnosis of PNH with hypoplastic bone marrow was made. Although treatment consisting of anabolic steroids and prednisolone was not effective, LDH activity was decreased during the clinical course. To evaluate the abnormal complement sensitive cells (PNH III cells) in the patient, complement lysis sensitivity test was repeated during the clinical course. The result indicated disappearance of the abnormal red cell populations and a change in the sensitivity to complement.
In early January 1986, the patient suffered from anal abscess, and died of cerebral hemorrhage on Febrary 16, 1986.

著者関連情報
© 1987 日本臨床血液学会
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