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プロブレムリスト
#1 鉄欠乏性貧血
#2 糖尿病
#3 小血管炎
#4 多発脳梗塞
多発脳梗塞⊃塞栓症⊃血栓⊃心原性
⊃大血管性
⊃奇異性
⊃菌塊
⊃腫瘍⊃粘液腫
⊃空気
⊃脂肪
⊃羊水
⊃血管炎
心臓腫瘤摘出術施行
・胸骨正中切開。
・心停止、完全体外循環施行。
・右房を切開し、卵円窩をくり抜くように腫瘤と一塊に
して摘出。牛心膜でくり抜いた卵円窩をパッチ形成。
・型通り体外循環終了し、閉創して終了
皮膚生検
PAS-アルシアンブルー染色
FactorⅧ免疫染色
プロブレムリスト
#1 鉄欠乏性貧血
#2 糖尿病
#3 小血管炎→(訂正)腫瘍塞栓症(#5)
#4 多発脳梗塞→脳塞栓症(#5)
#5 左房粘液腫
ATRIAL MYXOMA
Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine
Eleventh Edition
疫学
・80%以上が左房発生。次に右房、右室、左室の順。
・40-60歳で多い。
・男女比は1:3で女性に多い。
・ほとんどはsporadically。時に家族性(Carney complex)。
Etiology and Pathogenesis
・正確なorigin不明だが、卵円窩のsubendocardial cellsまたはmultipotential m
・85%は有茎性。
Clinical Manifestation
・無症状が最多。TTEで偶発的に見つかる。
・有症状なら、呼吸困難(特に右側臥位で悪化)や失神が多い。
・発熱、関節痛、筋痛、紅斑がみられることもある。それと塞栓症状。
・ESRやCRP、γGlbが上昇することもある。
・心雑音は64%でみられる。
(Pinede L, Duhaut P, and Loire R: Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore) 2001; 80
・最も多いのは収縮期雑音(50%)、次にⅠ音亢進(32%)、opening snap(26%)、
予後
・一般的に予後良好。
・再発率は5-14%。
・再発する時期は0.5-6.5年後。
その後の経過
・術後の経過は良好。
・その後は新たな皮疹・四肢疼痛・脱力なし。
・ステロイドは3週間かけて漸減し終了。
・現在は糖尿病で外来通院中。

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MGH GIM カンファレンス Case2-2

Editor's Notes

  1. 心臓腫瘤の病理(写真 3,4)では,一部に出血を伴う粘液様の間質を背景に円形・紡錘形の核を持つ細胞が索状~ 輪状構造を示しながら増生している。粘液腫と診断。
  2. 入院当日に圧痛のある紅斑部で施行した皮膚生検(写真 5,6,7,8)では,血管内皮は肥厚し多層化している。血管 内腔には単核の細胞集団と HE 染色で薄いピンクに染まる粘液様物を認める。
  3. この粘液様物は PAS アルシアンブルー染色で青く染まる。
  4. FactorⅧの免疫染色では,血管内腔の単核細胞が染まる。以上から粘液腫による腫瘍塞栓と診断した。