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090302 血液内科

●090302 血液内科

●貧血
「単位容積中のHb濃度の正常範囲を超えた現象」
→組織低酸素

血液伝票1枚で貧血は鑑別できる(8割)
・MCVは? (桁なんてどうでもいいから、計算!)
・造血は亢進?低下?  Ret
・ほかの血球は?  …造血幹細胞が壊れた?

 → これで、紹介すべきか自分で診るか、わかる

●MCVと発生機序で貧血は分類できる

     栄養障害   造血障害     破壊・喪失
小球性  鉄欠乏    サラセミア(非常にまれ))
            鉄がきゅうせい
            無トランスフェリン
             …

正球性         二次性貧血    急性失血
            再生不良性

大球性(140以上) 悪性貧血など(抗胃壁抗体・胃全摘)
   ビタミンB12(5年で尽きる)
   葉酸欠乏(妊娠、アルコール多飲)
    …妊娠中の葉酸欠乏はALLの危険因子

・フェリチンは炎症であがるので、鉄欠乏をマスクしうる

・鉄欠乏製貧血
「栄養障害の一表現型」
舌炎・さじ状爪
→ 自覚症状なし けど、全身倦怠感、集中力低下

中年以降は出血源の検索が最重要
輸血は原則不要
 例外 活動性の出血・重症心不全・高齢者・Hb3以下

 6以下のような重症は、たいてい癌

論文: 癌年齢の鉄欠乏あったとき、4人に1人は癌

フェロミア 4錠で十分
鉄の充足は3〜4ヶ月
錠剤で吐き気が出るなら、シロップ(含有量少ない)でたいていへいき

Hb10が目標
 2週間の静脈注射でたいていOK
あがってくるまでは5日くらいはタイムタグあり
 Retが3〜4日でどかんとあがってくる → 効いていると判断できる


B12 欠乏
 抹消神経障害だけでなく、痴呆のような形で出うる
   Hunter舌炎も
 背景を探る必要あり
 貧血度合いと比較し全身状態悪い
 ピロリ感染もあるかも
ちなみに、ピロリ除菌適応は、
ITPと大球性貧血
「メチコバール内服」もだいたい効く!!

葉酸欠乏
酒が原因なら、禁酒してまともに食事をすると改善
妊婦の葉酸欠乏は胎児奇形の原因に!


●発熱性好中球減少症
抗がん剤 = 毒
 好中球減少  → たいてい 緑膿菌やアスペルギルス
 液性免疫低下   肺炎球菌がやばい
 細胞性免疫低下

感染症が特定できるのは約3割
  熱が出たらとにかく抗生剤を、とやったら救命率上がった
数時間で勝負が決まる
 殺菌的抗生剤を極量使用
  (マクロライドとか及び出ない)
菌交代起こりやすい
好中球が回復すれば多くは軽快
 →2週間をとにかくしのぐ!

治療はリスク別に
呼吸機能 肝機能を評価しろ
 状態が悪いと熱もでないことあり

治療は、抗緑膿菌を「単独」で!
G-CSFはいまんとこよくわからない


当院の耐性
AMKは100%効く! ただ、十分量を使うのが難しい
 (保険量の倍!)
キノロンは8割
 PIPC 9割  〜おそらく一番信頼できる
 絶対に負けらないなら併用で

ペネムは耐性誘導が強い

緑膿菌のみがターゲットなら、TAZは必要ない

VCMはカテ感染あるなら…


G-SCF どう使うかは?
当院では
 悪性リンパ腫は 好中球1000未満で
  (1000以上あると、10000以上にドンとあがったりする)

72時間解熱なければ、深部真菌症を考える


●抗真菌剤
アスペルギルスは致命率100%
治療開始が遅れると、治癒は困難
確定診断できる状態は、たいてい手遅れ
  喀痰培養ででたときは、たいてい死亡…

ムコール
 アムホテリシンしか効かず、β-Dもでない

クリプトコッカス
 めったに診ない…


アムホテリシンB
 副作用が多くて十分量使えない…
 効くんだけれど

フルコナゾール
 アスペルギルス・ムコールには効かない
 副作用少ない

リポ化アムホテリシンB
 1日3万円…

ボリコナゾール
 実際には使いにくい
 高齢者の転倒やせん妄の誘引
 8割くらいが精神おかしくなる…

ただ、アスペルギルスは3ヶ月内服必要
内服1日4000円、点滴1日2万円強
  吸収とてもよく外来で治療を

イトラコナゾール
アスペルギルスにも効果(一部耐性)
カプセルは吸収悪い
内溶液は微妙な味…

ミカファンギン
 カンジダ・アスペルギルスにはとても効く
 副作用少ない
 注射しかできない


新規抗真菌剤 →アスペルギルスに勝てるようになった
  値段は高いが…


例: アスペルギルス
 肺動脈食い破って 天井まで喀血して即死

→ 大血管に隣接するなら外科的に


単剤無効時の併用療法

ファンガード、をどれか。
ほかを混ぜると打ち消しあう

 ただ、1日5万円…


カンジダ血症は
MCFGで!

・実際は、熱が出たら
 ガラクトマンナン併用で


●血小板減少症

・産生低下
 原疾患治療&輸血
・破壊・消費の亢進
 輸血は無駄
・偽性血小板減少 クエン酸チラートによる試験管内凝固
 血液像で確認できる
  ヘパリン採血で

発熱、凝固能低下
粘膜の紫斑は危険信号 「入院、輸血」
 内臓出血はとめられない!

慢性の血小板減少はいいが、
急性なら3万ではやばい!


●輸血
基準
Hb <7
Plt <1万  (DICは2万で 多めにしておく)
 症状、状態、年齢で前後
栄養障害の貧血で全身状態もちそうならO2投与


●ABO型
例外的に、生前から抗体を持っている


Minor mismatch
 患者血球に対する抗体が輸血に含まれている
 臨床的にはOK!

合併症
 最善は、輸血をしないこと
不適合輸血
 血管内溶血 → 血管内皮を障害
 多くは事務的なミス
免疫反応
 アナヒラキシー、喘息用発作 …だいたい15分で
 TRALI(輸血関連急性肺障害) 
  8割は3日で回復
  他人の白血球が原因に
   白血球除去フィルター使えばいいが赤字に
  (利尿剤は無効)

感染
HIV
 ウィンドウピリオドはすり抜けうる
 検査代わりの輸血はやばい!!

GVHD
 おきたら全員死ぬ 治療法なし
 HLAが似ていると起こしやすい(異物だと認識できないから)
 リンパ球を放射線照射で不活化できる
  微妙にKあがってくるので、腎不全は照射後すぐに

緊急時の対応
 輸血をやめる
 針はぬかない
 脱血 30〜50cc
  ルートの血を押し込むと悪化

・凝固優位のDIC(ほとんどのDIC)
感染症によるDICは血管内凝固優位
FDP上昇は軽度
FBG低下が炎症でマスクされる
 「正常範囲」は異常!!
  DICスコアに頼ると手遅れに
血小板の致命的な低下に注意
微小循環障害が主 腎不全、意識障害

TATが一番いいが、外注に…

トランサミンは禁忌

抗凝固療法なしの血小板輸血は禁忌!!!


・線溶優位のDIC
白血病によるDIC
FDP、D-dimerはどかんとあがる
血小板、FBGは低下
適切に補充しないと、死ぬ
出血したとおもったら、半日持たない

塞栓兆候なければトランサミンOK


抗凝固療法補充しながら血小板2〜3万を

ノンスロン 1500単位 重要


●多発性骨髄腫

60〜70でどかんとふえてくる
1万人に2〜3人


TTTはIgM、 ZTTはIgGを反映
 異常あれば注意
自己免疫疾患による多クローン性Igは鑑別
高Ca血症を疑う腎機能障害

疑ったらすぐに血液内科へ!!!
 腎不全起きたらておくれに!!!

…そうでなくても潜在性腎障害で、いろいろ薬使いにくくはなるが


●血液内科の発想法
・予測せよ そして備えよ
  過去のパターンから未来を予測する
  変化の方向と速度は?
    やばい速度でへってないか、回復してきてるか
  シナリオは3通り
   よくなった場合
   悪くなった場合
   すごく悪くなった場合
・リスクの重さは掛け算
  起こる可能性 X 起こった場合の重要性
・昨日と同じ明日は来ない


●薬剤性血小板減少
PIPC → 破壊性
精神科 → 産生障害?

ちなみに
高齢者増加率は75%異常
  国立社会保障 人口問題研究所
  日本の都道府県別将来推計人口

人間の生 という要求に限界はない
 医療費を絞って死を受容するのも道


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