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2.鍓嶉珨 B 娣嬪反姣嶇窗(x矛)鑳炴穻宸寸槫(B-LBL)锛氳垏鍓嶉珨 T 绱�(x矛)鑳炶叓鐦ょ浉鍙�锛屽墠 B 绱�(x矛)鑳炴(g貌u)鎴愮殑鑵槫 85%浠ヤ笂琛ㄧ従(xi脿n)鐐� ALL锛屽彧鏈� 10%锝� 20%鐐� LBL銆侭-LBL 鐧�(f膩)鐥呭勾榻¤純灏�锛屼腑浣嶅勾榻″皬浜� 6 姝层€傜櫦(f膩)鐥� 鐒�(w煤)鏄庨’鎬у垾鐗瑰緛銆傚父瑕�(ji脿n)琛ㄧ従(xi脿n)鐐烘穻宸寸祼(ji茅)鑵ぇ鍙婄毊鑶�銆佽粺绲勭箶(灏ゅ叾鏄牠闋搁儴)銆侀(甯歌(ji脿n)浜庤偂楠�銆佽剾楠�銆佽剨鏌遍)绛夌祼(ji茅)澶栦镜鐘�锛岃〃鐝�(xi脿n)鐐虹毊鑶氬鐧�(f膩)鎬х磪鑹茬祼(ji茅)绡€(ji茅)锛岄鍏�(n猫i)瀛ょ珛鎬ц叓濉婏紝褰卞儚瀛�(xu茅)妾㈡煡 绀烘憾楠ㄦ€ф垨纭寲鎬х梾璁�;灏戣(ji脿n)鐨勮〃鐝�(xi脿n)鏈夌副鑶�銆佽兏鑶滀镜鐘�锛屽収(n猫i)鑷熶镜鐘鑵庤嚐銆佹秷鍖栭亾娴告饯(r霉n)绛�銆備篃鏄撶櫦(f膩)鐢熼楂撳拰 CNS 娴告饯(r霉n)銆�
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鈶犳穻宸存瘝绱�(x矛)鑳炵殑妯�(bi膩o)蹇楋細鍖呮嫭 TdT銆丆D99銆丆D34銆丆D1a銆� LBL 渚�(l谩i)婧愪簬鍓嶉珨鐨� T 鎴� B 娣嬪反绱�(x矛)鑳�锛孴dT 琚獚(r猫n)鐐烘槸鍓嶉珨娣嬪反绱�(x矛)鑳炵殑鐗瑰緛鎬ф(bi膩o)蹇�锛岃兏鑵烘垨楠ㄩ珦鍓� T銆佸墠 B 绱�(x矛)鑳炲潎鍙憟鐝�(xi脿n)闄�(y谩ng)鎬ц〃閬�(d谩)锛屾枃鐛�(xi脿n)鍫�(b脿o)閬撳叾闄�(y谩ng)鎬х巼鍙仈(d谩) 90%锝�95%銆侰D99 涔熻瑾�(r猫n)鐐烘槸鍓� T 绱�(x矛)鑳炵殑鑹ソ妯�(bi膩o)瑷樼墿锛屼絾瀹冧笉鍍呰〃閬�(d谩)浜庝汉鐨勮兏鑵虹毊璩�(zh矛)绱�(x矛)鑳炰互鍙� ALL/LBL 绱�(x矛)鑳炰腑锛屽湪 Ewing's 鑲夌槫(ES)銆佸師濮嬬缍�(j墨ng)澶栬儦钁夎叓鐦�(PNET)浠ュ強鑳歌吅鐦や腑涔熷潎鏈夐櫧(y谩ng)鎬ц〃閬�(d谩)锛屽洜姝ゅ湪閼掑垾瑷烘柗涓婂繀闋堥厤浠ョ浉鎳�(y墨ng)鐨勫叾浠栨湁鏁堟(bi膩o)蹇楄伅(li谩n)鍚堣瀵�銆�
鈶-LBL 鐧�(f膩)鐢熶簬鑳歌吅绲勭箶锛岃叓鐦ょ窗(x矛)鑳炰締(l谩i)婧愪簬鍓嶈兏鑵虹窗(x矛)鑳炪€� 鑳歌吅绱�(x矛)鑳�锛岃〃閬�(d谩) CD3銆丆D2銆丆D5銆丆D7锛孋D7 鏄叾鐗圭暟鎬ф姉鍘�銆� 鐩墠瑾�(r猫n)鐐� T-LBL 渚�(l谩i)婧愪簬鑳歌吅涓櫦(f膩)鑲茬浉灏�(du矛)鎴愮啛鐨勫墠楂� T 绱�(x矛)鑳烇紝 鑰� T-ALL 杓冧箣鏈夋洿鏃╂湡鐨勫垎鍖栭殠娈�銆侰OG 鍫�(b脿o)鍛婁簡180 渚� T-LBL 鐨勫厤鐤〃鍨嬬壒寰佽垏 T-ALL 鐩镐技锛屼絾 CD4/CD8 闆欓櫧(y谩ng)鎬х殑鐧�(f膩)鐢熺巼鏄庨’楂樹簬鍚庤€�锛屼篃鏀寔 T-LBL 鍏锋湁鏇存垚鐔熷厤鐤〃鍨�銆�
鈶-LBL 鑵槫绱�(x矛)鑳炰締(l谩i)婧愪簬楠ㄩ珦锛屽爆浜庡墠鍓� B銆佸墠 B銆佹櫘閫� B 闅庢锛岃〃閬�(d谩) CD10銆丆D19銆丆D20銆丆D22銆丆D79a锛屽叾涓� CD79a 鐐� B 绱�(x矛)鑳炵壒鐣版€ф姉鍘�銆侻ayer 绛夊牨(b脿o)閬� 22%B-LBL 瀛樺湪鍏嶇柅鐞冭泲 鐧�γ杓曢張鐨勭己澶�锛岃€屽湪 B-ALL 涓殑鐧�(f膩)鐢熺巼鍍呯偤 1%锛屼篃鎻愮ず浜� B-LBL 渚�(l谩i)婧愪簬杓� B-ALL 绋嶆垚鐔熼殠娈电殑鍓嶉珨 B 绱�(x矛)鑳�銆�
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2.绱�(x矛)鑳為伜鍌冲(xu茅)(Cytogenetics)鍜屽垎瀛愮敓鐗╁(xu茅)(Molecular biology) 鏈梾鑸囧绋熀鍥犵暟甯告湁闂�(gu膩n)锛屽寘鎷姉鍘熷彈楂斿熀鍥�銆佹煋鑹查珨鐣板父銆佹姂鐧屽熀鍥犲け娲讳互鍙婄檶鍩哄洜鐨勬縺娲荤瓑銆傞€氶亷(gu貌)鏌撹壊楂旀牳鍨嬪垎鏋�銆丗ISH 鏂规硶銆佽瀺鍚堝熀鍥犲畾閲� RT-PCR銆両gH/TCR 閲嶆帓妾㈡脯(c猫)銆佹柊涓€浠f脯(c猫)搴�(NGS)鐨勬柟娉曢€�(j矛n)琛岀浉闂�(gu膩n)妾㈡煡銆�
95%浠ヤ笂 T-ALL/LBL 鏈� T 绱�(x矛)鑳炲彈楂�(T cell receptor, TCR)鍩哄洜鍏嬮殕鎬ч噸鎺�锛屽ぇ澶氭暩(sh霉) T-ALL 琛ㄧ従(xi脿n)鐐哄垎鍖栨垚鐔熼亷(gu貌)绋嬩腑棣栧厛閲嶆帓鐨� TCRγσ閺堝熀鍥�锛岃€� T-LBL 鍓囧鐐�αβ閺堝熀鍥犻噸鎺掞紝涔熸彁绀轰簡 T-ALL 鐨勫垎鍖栭殠娈垫棭浜� T-LBL銆傚ぇ绱� 20% T-ALL/LBL 鍚屾檪(sh铆)鏈� IgH 鍩哄洜閲嶆帓銆傜禃澶у鏁�(sh霉) B-LBL 鏈� Ig-H 鍩哄洜閲嶆帓锛屼篃鍙湁 Ig-L 鍩哄洜閲嶆帓銆� 50%锝�70%鐨� T-ALL/LBL 鏈夋煋鑹查珨鏍稿瀷鐣板父銆�
澶у鏁�(sh霉)鏍稿瀷鐣板父鑸囬潪闅ㄦ(j墨)鎬ф柗榛�(di菐n)鏈夐棞(gu膩n)锛氬父绱強 TCRασ(14q11.2)锛� β(7q35)锛�γ(7q14-15)锛屽寘鎷彃鍏ョ獊璁娿€佺己澶变互鍙婃煋鑹查珨鏄撲綅銆傛槗浣嶉€氬父浣垮師鐧屽熀鍥犳縺娲诲苟鐣板父琛ㄩ仈(d谩)锛屽皫(d菐o)鑷磋垏绱�(x矛)鑳炲懆鏈熻(di脿o)鎺у拰绱�(x矛)鑳炲垎鍖栫浉闂�(gu膩n)鐨勯澏鍩哄洜婵€娲昏€岃嚧鑵槫?锛孎(xi脿n)宸茬煡鑸� T-ALL/LBL 鐧�(f膩)鐥呮湁闂�(gu膩n)鐨勯噸瑕佸師鐧屽熀鍥犳湁 T 绱�(x矛)鑳炴€ユ€х櫧琛€鐥呭熀 鍥� 1(TAL-1)/SCL銆丠OX11銆丠OX11L2銆丩YL1銆丩MO1 鍜� LMO2 绛�銆�
del(9p)缂哄け灏�(d菐o)鑷寸殑鎶戠檶鍩哄洜 CDKN2A 鐨勪笩澶变篃鏄湰鐥呯櫦(f膩)鐢� 鐨勬(j墨)鍒朵箣涓€銆傜洰鍓嶅凡鐭ュ叿鏈夋縺娲荤獊璁婄疮鍙婄法纰煎墠 T 绱�(x矛)鑳炵櫦(f膩)鑲� 鐨勯棞(gu膩n)閸佃泲鐧界殑 NOTCH1 鍩哄洜鐨勭梾浜洪爯(y霉)鍚庤壇濂斤紝鐧�(f膩)鐢熺巼绱勭偤 50%; 鑰� 绱� 10% 鐨� 鐥� 浜� 鍏� 鏈� 鏌� 鑹� 楂� 6q 闆� 鍚� 鎬� 缂� 澶� ( Loss of heterozygosity at chromosome 6q;LOH6q)锛屽叾寰�(f霉)鐧�(f膩)棰�(f膿ng)闅�(xi菐n)澧� 楂�锛岄爯(y霉)鍚庝笉鑹�銆�
绱�60% B-ALL/LBL 鏈夌壒寰佹€ч伜鍌冲(xu茅)鏀硅畩涓斿叿鏈夐噸瑕佺殑鑷� 搴婅ê鏂峰拰闋�(y霉)鍚庡垽鏂锋剰缇�銆傞伜鍌冲(xu茅)涓婃彁绀洪爯(y霉)鍚庤純濂界殑鏈夎秴浜屽€� 楂�(鏌撹壊楂旀暩(sh霉)> 50)鏍稿瀷鍙� t(12;21)(p12;q22)鏄撲綅褰㈡垚TEL/AML1 铻嶅悎鍩哄洜;鑰屼簽浜屽€嶉珨(鏌撹壊楂旀暩(sh霉)灏忎簬 45)銆乼(9;22) (q34;q11.2) 褰� 鎴� BCR/ABLl 銆� t ( 4;11 ) 鎴� MLL/AF4 銆� t (1;19)(q23;13.3)褰㈡垚鐨�(E2A-PBX1; TCF3/PBX1)浠ュ強 Ph 妯e熀鍥犳垨绐佽畩绛夎垏鑷ㄥ簥鐧傛晥涓嶄匠銆侀爯(y霉)鍚庝笉鑹浉闂�(gu膩n)銆�
3.琛€甯歌(gu墨)锛氱櫧绱�(x矛)鑳炲彲姝e父鎴栬紩搴﹀崌楂�锛屽彲鏈夎钵琛€锛屽鐐� 姝g窗(x矛)鑳炴鑹茬礌鎬ц钵琛€銆傜暥(d膩ng)楠ㄩ珦鍙楃疮鏅�(sh铆)锛屽彲鏈夌櫧绱�(x矛)鑳炵附鏁�(sh霉)鍗囬珮鎴栨笡浣�锛屽鍛ㄨ鍑虹従(xi脿n)骞肩绱�(x矛)鑳�锛屽彲浼存湁璨ц鍜�/鎴栬灏忔澘娓涗綆銆�
4.琛€鐢熷寲锛氳倽鑵庡姛鑳�銆佷钩閰歌劔姘叾(LDH)銆侀浕瑙h唱(zh矛)鏄繀鏌ラ爡(xi脿ng)鐩�銆傚線寰€琛ㄧ従(xi脿n)鐐哄翱閰搞€佷钩閰歌劔姘叾澧為珮锛岄€欏叐闋�(xi脿ng)鎸囨(bi膩o)灏�(du矛) 鐤剧梾绶╄В鎯呮硜鍙婇爯(y霉)鍚庢湁鎻愮ず浣滅敤銆�
5.鍑濊鍔熻兘锛氬寘鎷� PT銆丄PTT銆乀T銆丗IB銆丏 浜岃仛楂斻€丗DP銆� 鏈梾鍙€犳垚鍑濊鍔熻兘鐣板父锛屽嚌琛€閰跺師鍜岀簴缍泲鐧藉師娓涘皯锛屽緸鑰屽皫(d菐o)鑷村嚌琛€閰跺師鏅�(sh铆)闁撳欢闀�(zh菐ng)鍜屽嚭琛€銆�
6.楠ㄩ珦妾㈡煡锛氶楂撳彈绱檪(sh铆)锛岄楂撲腑骞肩娣嬪反绱�(x矛)鑳炰竴鑸皬 浜� 25%锛岄€氶亷(gu貌)娴佸紡绱�(x矛)鑳炶(sh霉)銆佺窗(x矛)鑳為伜鍌冲(xu茅)銆佸熀鍥犻噸鎺掔瓑妾㈡煡鏈� 鍔╀簬瑷烘柗鍜屽垎鍨嬨€傛祦寮忕窗(x矛)鑳炲剙妾㈡脯(c猫)鍙互鐧�(f膩)鐝�(xi脿n)闅辨€х殑楠ㄩ珦鍙楃疮銆� 楠ㄩ珦娲绘灏�(du矛)楠ㄩ珦渚电姱鐨勬鍑虹巼楂樹簬楠ㄩ珦褰㈡厠(t脿i)瀛�(xu茅)銆�
7.鑵﹁剨娑叉鏌ワ細甯歌(gu墨)琛岃叇鑴婃恫甯歌(gu墨)銆佺敓鍖�銆佺敥鐗囨壘鑵槫 绱�(x矛)鑳炰簡瑙f湁鐒�(w煤)涓绁炵稉(j墨ng)绯荤当(t菕ng)(Central nervous system, CNS) 鐨勪镜鐘€傛祦寮忕窗(x矛)鑳炲剙妾㈡脯(c猫)鑵槫绱�(x矛)鑳炲厤鐤垎鍨嬪彲鎻愰珮 CNS 渚电姱 妾㈠嚭鐜�銆�
8.褰卞儚瀛�(xu茅)妾㈡煡锛氭不鐧傚墠闇€甯歌(gu墨)閫�(j矛n)琛屽叏韬奖鍍忓(xu茅)妾㈡煡浠ユ槑 纰哄彈绱儴浣�锛屽崝(xi茅)鍔╁垎鏈�銆俋 绶氭鏌ワ細楠ㄧ墖(鏈夐銆侀棞(gu膩n)绡€(ji茅)鑵棝 - 7 - 鑰�);B 瓒咃細鑷冲皯鍖呮嫭闋搁儴娣嬪反绲�(ji茅) B 瓒呫€佽吂閮� B 瓒呭強鐥呯伓閮ㄤ綅 鐩搁棞(gu膩n) B 瓒�(鍋氭渶澶х槫鐏舵脯(c猫)閲�);CT锛氳嚦灏戝寘鎷兏鑵圭泦 CT 骞虫巸 (鑻ヤ綔 PET/CT锛屽彲涓嶅仛甯歌(gu墨) CT 妾㈡煡锛屽儏浣滅梾鐏堕儴浣嶆鏌�); 鐥呯伓閮ㄤ綅闇€琛屽寮�(qi谩ng) CT 妾㈡煡;鎳风枒涓绁炵稉(j墨ng)绯荤当(t菕ng)鐥呰畩鐨勮闋”鍜�(鎴�)鑴婇珦 MRI 妾㈡煡;鏈夋浠惰€呯洝鍙兘琛屽叏韬� PET/CT 妾㈡煡銆�
(涓�)鍏掔 LBL 鐨勮ê鏂锋(bi膩o)婧�(zh菙n)
1.瑷烘柗妯�(bi膩o)婧�(zh菙n) WHO 2016鏈夐棞(gu膩n)娣嬪反閫犺绲勭箶鑵槫鍒嗛涓(gu墨)瀹氫簡LBL鐨勮ê 鏂锋(bi膩o)婧�(zh菙n)銆傞櫎渚濇摎(j霉)鎮h€呰嚚搴婄壒榛�(di菐n)澶�锛屽潎闇€缍�(j墨ng)閬�(gu貌)鍙楃疮绲勭箶娲绘锛� 閫�(j矛n)琛岀祫绻旂梾鐞嗗(xu茅)銆佸厤鐤〃鍨�銆佺窗(x矛)鑳為伜鍌冲(xu茅)鍜屽垎瀛愮敓鐗╁(xu茅)鐨勬 娓�(c猫)纰鸿ê銆傝嚚搴婂垎鏈燂細鏍规摎(j霉)鍏ㄨ韩褰卞儚瀛�(xu茅)銆佽叇鑴婃恫鍙婇楂撶瓑妾㈡煡锛屼互 寰€鎸夌収St Jude鍒嗘湡绯荤当(t菕ng)纰哄畾鍒嗘湡(瑕�(ji脿n)闄勮〃1)銆�
闅ㄨ憲閱�(y墨)瀛�(xu茅)鐧�(f膩) 灞�锛屽厭绔HL鏂扮殑鐥呯悊浜炲瀷鐨勫鍔�锛岀窗(x矛)鑳為伜鍌冲(xu茅)銆佸垎瀛愮敓鐗� 瀛�(xu茅)鍜屽厤鐤〃鍨嬫柟闈㈢殑閫�(j矛n)姝ワ紝鏂扮殑瑷烘柗鏂规硶鍦ㄦ娓�(c猫)寰皬鎿�(ku貌)鏁g梾鐏舵垨寰皬娈樼暀鐥呯伓鐨勮嚚搴婃噳(y墨ng)鐢ㄤ互鍙婂奖鍍忓(xu茅)鏂归潰鐨勯噸瑕侀€�(j矛n)灞�锛� 鍦ㄥ師鏈塖t Jude 鍒嗘湡绯荤当(t菕ng)涓婇€�(j矛n)琛屼簡瑁�(b菙)鍏呭拰鏀硅壇锛岀敘(ch菐n)鐢熶簡鏂扮殑鍒嗘湡绯荤当(t菕ng)锛氫慨瑷傚湅(gu贸)闅涘厭绔HL鍒嗘湡绯荤当(t菕ng)(IPNHLSS)锛岄€�(j矛n)涓€姝ユ槑纰哄拰瑁�(b菙)鍏呬簡姣忎竴鏈熺殑瀹氱京锛屽苟灏囨渶鏂扮殑閱�(y墨)瀛�(xu茅)閫�(j矛n)灞曚俊鎭姞鍏ヤ簡 鍒嗘湡绯荤当(t菕ng)(瑕�(ji脿n)闄勮〃2)銆�
2.楠ㄩ珦渚电姱鐨勮ê鏂凤細闇€瑕佽鍏╁€�(g猫)閮ㄤ綅楠ㄩ珦绌垮埡鍜岃嚦灏戜竴 鍊�(g猫)閮ㄤ綅楠ㄩ珦娲绘閫�(j矛n)琛屽垎鏋愬畾缇╅楂撲镜鐘�銆�
(1)楠ㄩ珦绌垮埡绱�(x矛)鑳炲舰鎱�(t脿i)瀛�(xu茅)锛氶楂撳辜绋氱窗(x矛)鑳炴垨娣嬪反鐦ょ窗(x矛)鑳�≥5%銆�
(2)楠ㄩ珦鍏嶇柅琛ㄥ瀷鏂规硶(鍏嶇柅绲勭箶鍖栧(xu茅)鎴栨祦寮忕窗(x矛)鑳炶(sh霉)鍒嗘瀽锛氱壒鎸囨穻宸寸槫绱�(x矛)鑳炵櫨鍒嗘瘮)闄�(y谩ng)鎬�銆� (3)楠ㄩ珦绱�(x矛)鑳為伜鍌冲(xu茅)鎴朏ISH鍒嗘瀽闄�(y谩ng)鎬�銆�
(4)楠ㄩ珦PCR鏂规硶妾㈡脯(c猫)鍒嗗瓙鐢熺墿瀛�(xu茅)闄�(y谩ng)鎬�銆�
(5)楠ㄩ珦娲绘鍙互瑕�(ji脿n)鍒拌叓鐦ょ窗(x矛)鑳炴蹈娼�(r霉n)銆�
3.涓绁炵稉(j墨ng)绯荤当(t菕ng)渚电姱鐨勮ê鏂凤細鑵﹁剨娑茬媭鎱�(t脿i)鍒嗙礆(j铆)涓昏渚濇摎(j霉) 鑷ㄥ簥琛ㄧ従(xi脿n)銆佸奖鍍忓(xu茅)鏀硅畩銆佽叇鑴婃恫妾㈡煡銆�
(1) CNS1锛氬悓鏅�(sh铆)绗﹀悎浠ヤ笅涓夋锛氣憼鑵﹁剨娑蹭腑鐒�(w煤)鑵槫绱�(x矛) 鑳�;鈶$劇(w煤)涓绁炵稉(j墨ng)绯荤当(t菕ng)鐣板父鐨勮嚚搴婅〃鐝�(xi脿n);鈶㈢劇(w煤)涓绁炵稉(j墨ng)绯荤当(t菕ng) 鐣板父鐨勫奖鍍忓(xu茅)(CT/MRI)渚濇摎(j霉)銆�
(2) CNS2锛氱鍚堜互涓嬩换浣曚竴姊濓細
鈶犺叞绌跨劇(w煤)鎼嶅偡—鑵﹁剨 娑蹭笉娣疯(绱呯窗(x矛)鑳�:鐧界窗(x矛)鑳�≤100:1)鏅�(sh铆)锛岃叇鑴婃恫鐧界窗(x矛)鑳炶▓(j矛)鏁�(sh霉) ≤5/μl锛屽苟瑕�(ji脿n)鍒版槑纰虹殑鑵槫绱�(x矛)鑳�;
鈶¤叞绌挎湁鎼嶅偡—鑵﹁剨娑叉贩 琛€(绱呯窗(x矛)鑳�:鐧界窗(x矛)鑳�>100:1)鏅�(sh铆)锛岃叇鑴婃恫瑕�(ji脿n)鍒版槑纰虹殑鑵槫绱�(x矛) 鑳�;
鈶㈣叞绌挎湁鎼嶅偡骞剁偤琛€鎬ц叇鑴婃恫锛屼笉璜栨湁鐒�(w煤)鑵槫绱�(x矛)鑳�锛屽鍒濊ê鐧界窗(x矛)鑳炴暩(sh霉)>50×10 9/L 鍓囨鐐� CNS2;
鈶d腑妯炶嚚杩戦儴浣嶇殑 渚电姱鐐虹櫦(f膩)鐢� CNS 渚电姱鐨勫嵄闅�(xi菐n)鍥犵礌锛岀偤浜嗗姞寮�(qi谩ng)闋�(y霉)闃叉鐐� CNS2锛� 闋潰閮ㄧ毊鑶氥€佽粺绲勭箶鎴栭楠间镜鐘�锛屽椤遍銆侀蓟绔�銆佺溂鐪剁瓑(鏈� 绐佺牬纭叇鑶�);鑴婃煴楠ㄧ牬澹炴垨妞庢梺渚电姱涓嶄即绁炵稉(j墨ng)绯荤当(t菕ng)鐧ョ媭鐨�锛岀劇(w煤) CNS 鍗犱綅銆佽叇鑴婃恫妾㈡煡姝e父銆�
(3)CNS3锛氱鍚堜互涓嬩换浣曚竴姊濈偤 CNS 渚电姱锛�
鈶犺叇鑴婃恫鐧� 绱�(x矛)鑳炶▓(j矛)鏁�(sh霉)>5/μl锛屽苟浠ヨ叓鐦ょ窗(x矛)鑳炵偤涓伙紝鍚屾檪(sh铆)绱呯窗(x矛)鑳�:鐧界窗(x矛)鑳�≤100:1;鎴栬€呰叇鑴婃恫鐧界窗(x矛)鑳炶▓(j矛)鏁�(sh霉)>5/μl锛屽叾涓叓鐦ょ窗(x矛)鑳炴墍 鍗犳瘮渚嬮珮浜庡鍛ㄨ骞肩绱�(x矛)鑳炵櫨鍒嗘瘮;
鈶¢”绁炵稉(j墨ng)楹荤椆锛屽嵆浣胯叇鑴婃恫涓劇(w煤)鑵槫绱�(x矛)鑳�銆侀”鑵� MRI/CT 鏈櫦(f膩)鐝�(xi脿n)鍗犱綅鎬х梾璁�;
鈶� CT/MRI 鍙(ji脿n)鑵�銆佽剨楂撴垨鑵﹁啘銆佽剨鑶滅梾璁�銆�
鈶h剨鏌遍鐮村鎴栨 鏃佷镜鐘紩璧锋帓灏跨暟甯�銆佽偄楂旀椿鍕�(d貌ng)闅滅绛夌缍�(j墨ng)绯荤当(t菕ng)鐧ョ媭銆�
4.鐫父渚电姱鐨勮ê鏂凤細鎮e厭琛ㄧ従(xi脿n)鐐虹潽涓稿柈鍋�(c猫)鎴栭洐鍋�(c猫)鑵ぇ锛岃唱(zh矛)鍦拌畩纭垨鍛堢祼(ji茅)绡€(ji茅)鐙€缂轰箯褰堟€ф劅锛岄€忓厜瑭﹂(y脿n)闄版€�锛孊 瓒呮鏌� 鍙櫦(f膩)鐝�(xi脿n)鐫父鍛堥潪鍧囪唱(zh矛)鎬ф蹈娼�(r霉n)鐏�锛屽垵瑷烘偅鍏掑彲涓嶄簣娲绘銆傚湪鍏� 韬寲鐧傜珐瑙g殑鎮e厭鍑虹従(xi脿n)鐫父鑵ぇ鑰�锛屾噳(y墨ng)閫�(j矛n)琛屾椿妾互纰哄畾鏄惁 鐫父寰�(f霉)鐧�(f膩)銆�
(鍥�)閼掑垾瑷烘柗
1.鎬ユ€ч珦绯荤櫧琛€鐥呭拰绮掔窗(x矛)鑳炶倝鐦わ細褰㈡厠(t脿i)瀛�(xu茅)涓婃垚楂撶窗(x矛)鑳炶垏 LBL 鐨勬穻宸存瘝绱�(x矛)鑳為洠浠ュ崁(q奴)鍒�銆備絾鎴愰珦绱�(x矛)鑳炲舰鎱�(t脿i)杓冧笉涓€鑷�锛岀窗(x矛) 鑳炶唱(zh矛)杓冨锛岃優璩�(zh矛)鍏�(n猫i)鍙(ji脿n)绱呮煋妫掔媭 Auer 姘忓皬楂�銆傚厤鐤〃鍨� MPO銆� CD13銆丆D14銆丆D15 鍜� CD64 鍛堥櫧(y谩ng)鎬у弽鎳�(y墨ng);TdT 浠ュ強 T 鎴� B 娣嬪反绱�(x矛)鑳炴(bi膩o)蹇楃偤闄版€с€�
2.Burkitt’s 娣嬪反鐦わ細褰㈡厠(t脿i)瀛�(xu茅)涓婁簩鑰呮湁鐩镐技涔嬭檿锛屽潎鐐� 涓瓑澶у皬鐨勫柈褰㈡€ц叓鐦ょ窗(x矛)鑳炴(g貌u)鎴�锛岄兘鍙嚭鐝�(xi脿n)“鏄熺┖鐝�(xi脿n)璞�”銆備絾 LBL 鍋惰(ji脿n)鐏舵€�“鏄熺┖鐝�(xi脿n)璞�”锛岃€� Burkitt’s 娣嬪反鐦�“鏄熺┖鐝�(xi脿n) 璞�”甯稿父璨┛浜庢暣鍊�(g猫)鐦ょ祫绻�銆傜槫绱�(x矛)鑳炶〃閬�(d谩) sIgM銆丆D10銆丒BER 1/2 鍘熶綅闆滀氦闄�(y谩ng)鎬�銆傜窗(x矛)鑳為伜鍌冲(xu茅)椤ず鏈夌壒寰佹€� t(8;14) (q23;q21)鎴� c-myc 鍩哄洜閲嶆帓銆�
3.闈炴穻宸撮€犺绯荤当(t菕ng)灏忕窗(x矛)鑳炴€ф儭鎬ц叓鐦わ細涓昏鍖呮嫭灏ゆ枃姘忚倝鐦�銆佸師濮嬬缍�(j墨ng)澶栬儦钁夎叓鐦ゃ€佺缍�(j墨ng)姣嶇窗(x矛)鑳炵槫銆佸皬绱�(x矛)鑳炴湭鍒嗗寲鐧岀瓑銆傚挨鍏舵槸鐧�(f膩)鐢熶簬娣嬪反绲�(ji茅)澶栫殑灏忓湏绱�(x矛)鑳炶叓鐦�锛岀敱浜庡叾姣嶇窗(x矛) 鑳炲寲鐗瑰緛锛屼笖鍏掔浜︾偤濂界櫦(f膩)骞撮健绲勶紝鍥犳鐣�(d膩ng) CD99(+)锛岃€� LCA銆� CD3銆丆D20 鍧囬櫚鎬ф檪(sh铆)鏄撹瑷虹偤姝ら鐤剧梾?锛熻泧x鐢ㄥ绋姉鍘�锛� 鍖呮嫭绁炵稉(j墨ng)鍏�(n猫i)鍒嗘硨妯�(bi膩o)瑷樼墿(CgA銆丼yn)绛夊彲璩囬憭鍒�銆�
4.鑳歌吅鐦わ細涓€鑸綅浜庡墠涓婄副闅�锛屾サ灏戠櫦(f膩)鐢熶簬鍏掔鍙婇潚灏� 骞�銆傚緸鐥呯悊涓婂叐鑰呭潎琛ㄧ従(xi脿n)鐐烘穻宸寸窗(x矛)鑳炲綄婕€х敓闀�(zh菐ng)锛孴dT 鍧囧憟 闄�(y谩ng)鎬�銆傝兏鑵虹槫鐦ょ祫绻斿憟鍒嗚憠鐙€锛岃(ji脿n)鏄庨’鐨勭簴缍寘鑶滃強绮楀ぇ鐨� 绾栫董闁撻殧;鏁e湪鐨勪綆鍒嗗瓙璩�(zh矛)閲忚铔嬬櫧(CK)闄�(y谩ng)鎬х窗(x矛)鑳炶搏绌夸簬 鏁村€�(g猫)鐦ょ祫绻�銆傝€� LBL 涓�锛岀敱浜庣槫绱�(x矛)鑳炵殑娴告饯(r霉n)鎬х敓闀�(zh菐ng)锛屽湪娈樺瓨鐨勮兏鑵虹祫绻斾腑鍙嚭鐝�(xi脿n)灏戦噺鐏舵€у垎甯冪殑 CK 闄�(y谩ng)鎬х窗(x矛)鑳�銆�
5.鐣歌儙鐦わ細绺遍殧鍏�(n猫i)鍙兘鐧�(f膩)鐢熻壇鎬у拰鎯℃€х暩鑳庣槫锛屼篃澶氱櫦(f膩) 鐢熶簬鍓嶇副闅�锛岃叓鐦ゅ収(n猫i)鍚湁楂樺瘑搴︾殑鐗╄唱(zh矛)濡傞垼鍖栬粺楠�銆侀銆侀綊绛夌祫绻旀檪(sh铆)锛孹 绶氭鏌�?锛熺罕_瀹氳ê鏂�;琛€涓敳鑳庤泲鐧藉楂樿€呭鐐烘儭鎬�銆�
6.鍏朵粬绺遍殧鑵槫锛氬绁炵稉(j墨ng)婧愭€ц叓鐦�銆佺簴缍槫銆佽剛鑲槫銆� 娣嬪反绠$槫绛�锛屽潎闇€閫氶亷(gu貌)鑷ㄥ簥琛ㄧ従(xi脿n)銆佽叓鐦ゅ湪绺遍殧涓殑浣嶇疆銆佸奖鍍忓(xu茅)鐗归粸(di菐n)鍙婄梾鐞嗘鏌ラ€�(j矛n)琛岄憭鍒�銆�
(浜�)鑷ㄥ簥娌荤檪鍙嶆噳(y墨ng)瑭�(p铆ng)浼版(bi膩o)婧�(zh菙n)銆佽⿻(p铆ng)浼版檪(sh铆)闁撻粸(di菐n)鍙婂収(n猫i)瀹�
1. 瑭�(p铆ng)浼版(bi膩o)婧�(zh菙n)
(1)鐦ょ伓绶╄В鎯呮硜
鈶犲畬鍏ㄧ珐瑙�(CR)锛氶€氶亷(gu貌)鏌ラ珨鍜屽奖鍍忓(xu茅)妾㈡煡璀夊(sh铆)鎵€鏈夌槫鐏跺叏閮ㄦ秷澶便€侰T 椤ず鍘熻叓澶х殑娣嬪反绲�(ji茅)鍜岃叓濉婂凡绺皬鑷虫甯歌寖鍦嶆渶澶у緫绶�< 1.5 cm)锛屽悇鍣ㄥ畼褰卞儚瀛�(xu茅)鍙(ji脿n)鍒扮殑鎵€鏈夌槫鐏跺潎 宸叉秷澶便€侾ET 鎺冩弿宸茬劇(w煤)闄�(y谩ng)鎬х櫦(f膩)鐝�(xi脿n)銆傞楂撴秱鐗�/娲绘姝e父;娴佸紡 绱�(x矛)鑳炶(sh霉)閫�(j矛n)琛� MRD 鐩�(ji膩n)娓�(c猫)鐐洪櫚鎬�銆�
鈶℃湭纰哄畾鐨勫畬鍏ㄧ珐瑙�(Unconfirmed complete response)锛� 闆栫劧鐥呯伓鏈€澶у緫>1.5cm锛屼絾绺介珨涓婄梾鐏剁府灏�>75%(鐩镐簰鍨� 鐩村叐姊濇渶澶ф┇寰�)銆傚緸褰卞儚瀛�(xu茅)涓婄劇(w煤)娉曠⒑瀹氭槸鍚︾偤娈樼暀鐨勮叓鐦ゆ€� 鐥呯伓锛屾噳(y墨ng)琛岀梾鐞嗘椿妾㈢⒑瀹�锛岃€屾湁鐨勭梾鐏跺張鍙兘涓嶉仼浜庢墜琛�(sh霉)娲绘纰哄畾銆�
鈶㈤儴鍒嗙珐瑙� (PR)锛氱槫鐏舵渶澶у緫绶氱府灏�≥ 50% 锛屾矑(m茅i)鏈夋柊 鐧�(f膩)鐦ょ伓
鈶g劇(w煤)鍙嶆噳(y墨ng)(NR)(鐤剧梾绌�(w臎n)瀹�)锛氳⿻(p铆ng)浼伴’绀烘矑(m茅i)鏈夐仈(d谩)鍒� PR锛� 浣� 涔熸矑(m茅i)鏈夋柊鐧�(f膩)鐦ょ伓銆�
鈶ょ柧鐥呴€�(j矛n)灞� (PD)锛氫换浣曠槫鐏跺澶�≥ 25% 鎴栧嚭鐝�(xi脿n)鏂扮櫦(f膩)鐦� 鐏舵垨楠ㄩ珦寰�(f霉)鐧�(f膩)銆�
(2)鍒嗗瓙鐢熺墿瀛�(xu茅)绶╄В(MCR)锛氱洠(ji膩n)娓�(c猫)鍒嗗瓙鐢熺墿瀛�(xu茅)妯�(bi膩o)瑷�锛屼緥濡傞€氶亷(gu貌) PCR 鏂规硶鐩�(ji膩n)娓�(c猫)楠ㄩ珦铻嶅悎鍩哄洜寰為櫧(y谩ng)鎬ц綁(zhu菐n)鐐洪櫚鎬�銆�
(3)鍒嗗瓙鐢熺墿瀛�(xu茅)寰�(f霉)鐧�(f膩)(鎴栭櫚杞�(zhu菐n)闄�(y谩ng))锛氬鏈夊垎瀛愮敓鐗╁(xu茅)妯�(bi膩o) 瑷�锛屾不鐧傚悗杞�(zhu菐n)鐐洪櫚鎬х殑鍩哄洜鎴栧叾浠栬瀺鍚堝熀鍥犲啀娆℃娓�(c猫)鐐洪櫧(y谩ng)鎬�銆�
2.鍖栫檪鏈熼枔瑭�(p铆ng)浼版檪(sh铆)闁撻粸(di菐n)
(1)寮�(qi谩ng)鐨勬澗闋�(y霉)娌荤檪 d8锛氳⿻(p铆ng)浼板鍛ㄨ骞肩绱�(x矛)鑳炴暩(sh霉)鍙婁富瑕佺槫鐏�;
(2)VDLP 鏂规瑾樺皫(d菐o)娌荤檪 d15 鍍呬綔楠ㄩ珦甯歌(gu墨)鍙� MRD 瑭�(p铆ng)浼�;
(3)VDLP 鏂规瑾樺皫(d菐o)娌荤檪 d33;
(4)CAM2 鏂规鍖栫檪鍚庯細绋辩偤涓湡瑭�(p铆ng)浼�;
(5)寤堕伈寮�(qi谩ng)鍖栤厾闁嬪鍓�;
(6)寤堕伈寮�(qi谩ng)鍖栤叀闁嬪鍓�;
(7)缍寔娌荤檪鏈熼枔锛氱董鎸佹不鐧傞枊濮嬫檪(sh铆)锛氬寘鎷腑闁撶董鎸佸強 澶х董鎸侀枊濮嬪墠;缍寔娌荤檪鍓嶅洓鍊�(g猫)寰挵(hu谩n)姣� 8 鍛ㄨ⿻(p铆ng)浼颁竴娆�(鍖呮嫭 楠ㄩ珦妾㈡煡鍙婄槫鐏惰⿻(p铆ng)浼�)锛屼互鍚庢瘡 8 鍛ㄥ皬瑭�(p铆ng)浼颁竴娆�(绨�(ji菐n)鍠奖鍍忓(xu茅)妾㈡煡锛岀劇(w煤)楠ㄧ┛鍜岃叞绌�)锛屾瘡 16 鍛ㄥぇ瑭�(p铆ng)浼� 1 娆�(鍖呮嫭鑵﹁剨娑插拰楠ㄩ珦妾㈡煡)銆�
3.鍖栫檪閬�(gu貌)绋嬩腑瑭�(p铆ng)浼板収(n猫i)瀹�
(1)寮�(qi谩ng)鐨勬澗闋�(y霉)娌荤檪 d8锛氶€�(j矛n)琛屽鍛ㄨ骞肩绱�(x矛)鑳炶⿻(p铆ng)浼板強涓昏鐦ょ伓瑭�(p铆ng)浼�锛屼互鍋氫富瑕佺槫鐏剁殑 B 瓒呮鏌ョ偤涓�锛岀副鑶堝崰浣嶅彲琛� CT 妾㈡煡锛屽苟琛岃叓鐦ゅ緫绶氭脯(c猫)閲�锛岃叓鐦ゅ洖绺ぇ浜� 25%銆佸鍛ㄨ骞� 绋氱窗(x矛)鑳炲皬浜� 1000/mm³鐐哄皪(du矛)寮�(qi谩ng)鐨勬澗鏁忔劅;
(2)VDLP 鏂规 d15锛氭湁楠ㄩ珦渚电姱鐨勭梾浜哄京(f霉)鏌ラ楂撳舰鎱�(t脿i)锛� 骞堕€氶亷(gu貌)娴佸紡绱�(x矛)鑳炲剙妾㈡脯(c猫)楠ㄩ珦 MRD;
(3)鍏朵粬鏅�(sh铆)闁撻粸(di菐n)瑭�(p铆ng)浼帮細鍖呮嫭鈶犻楂撶珐瑙f儏娉侊細楠ㄩ珦褰㈡厠(t脿i)鍜屾祦寮忕窗(x矛)鑳炲剙妾㈡脯(c猫)楠ㄩ珦 MRD;楠ㄩ珦铻嶅悎鍩哄洜闄�(y谩ng)鎬ц€呴渶寰�(f霉)鏌ラ 楂撳熀鍥犺綁(zhu菐n)闄般€傗憽鐦ょ伓鎯呮硜锛氱槫鐏堕儴浣嶇浉闂�(gu膩n)褰卞儚瀛�(xu茅)妾㈡煡銆�
鍥涖€佽嚚搴婂嵄闅�(xi菐n)搴﹀垎灞�
LBL 鍗遍毆(xi菐n)搴﹀垎灞ゅ皻鐒�(w煤)绲�(t菕ng)涓€妯�(bi膩o)婧�(zh菙n)锛屽師鍓囦笂鏄稖鍚堣嚚搴婂垎鏈�銆� 鑵槫绱�(x矛)鑳為伜鍌冲(xu茅)鐗瑰緛浠ュ強娌荤檪鍙嶆噳(y墨ng)绛夊姞浠ョ⒑瀹氥€傚湪娌荤檪鏃╂湡闇€鏍规摎(j霉)鍗遍毆(xi菐n)鍥犵礌锛岀⒑瀹氬嵄闅�(xi菐n)搴﹀垎绲勭偤浣庡嵄绲�銆佷腑鍗辩祫銆侀珮鍗� 绲�銆�(涓€)浣庡嵄绲�
鎸夌収淇▊鐨勫湅(gu贸)闅涘厭绔� NHL 鍒嗘湡绯荤当(t菕ng)锛屼笉鍏锋湁楂樺嵄鍥犵礌鐨� 鈪�銆佲叀鏈熺梾浜�(瀛樺湪鏃╂湡鑵槫鑷櫦(f膩)婧惰В鎴栧法澶х槫濉婄殑鈪℃湡鐥� 浜洪櫎澶�)銆�
(浜�)涓嵄绲�
涓嶅叿鏈夐珮鍗卞洜绱犵殑鈪€€佲叄鏈熺梾浜�銆�
(涓�)楂樺嵄绲�
闇€閫氶亷(gu貌)鐦ょ伓銆侀楂�銆佸鍛ㄨ瑭�(p铆ng)浼颁簡瑙f不鐧傚弽鎳�(y墨ng)銆�
1.寮�(qi谩ng)鐨勬澗闋�(y霉)娌荤檪 d8锛屽鍛ㄨ骞肩绱�(x矛)鑳炲ぇ浜� 1000/mm³銆�
2.瑾樺皫(d菐o)娌荤檪 d15 楠ㄩ珦骞肩绱�(x矛)鑳�>25%銆�
3.瑾樺皫(d菐o)娌荤檪 d33 鑵槫娈樺瓨>25%鎴栭楂撳辜绋氱窗(x矛)鑳�>5%;楠� 楂� MRD≥10 -2 ;鑵﹁剨娑蹭腑鎸佺簩(x霉)瀛樺湪骞肩绱�(x矛)鑳�(鎸囦笁娆¢灅娉ㄥ悗鑵� 鑴婃恫涓粛鏈夎叓鐦ょ窗(x矛)鑳�)銆傚叾涓换涓€闋�(xi脿ng)銆�
4.闉忓浐娌荤檪鍓�(CAM2 鍖栫檪鍚�)瑭�(p铆ng)浼颁粛鏈夋畼鐣欑梾鐏惰€呯洝閲忚娲绘锛屼粛鐐鸿叓鐦ょ祫绻�(濡傛灉娌�(m茅i)鏈夋浠跺仛浜屾娲绘鍙姊濅欢 琛� PET/CT 鍗�(xi茅)鍔╅憭鍒�);楠ㄩ珦 MRD≥10 -3銆傚叾涓换涓€闋�(xi脿ng)銆�
5.鍏锋湁涓嶈壇閬哄偝瀛�(xu茅)鐗瑰緛锛歵(9;22)鎴� BCR/ABL锛宼(4;11) 鎴朚LL/AF4鍙婂叾浠朚LL鍩哄洜閲嶆帓銆丳h妯LL鐩搁棞(gu膩n)鍩哄洜濡侷KZF1銆� CRLF2銆丣AK2 绛�銆�
浜斻€佹不鐧�
(涓€)绯荤当(t菕ng)鍖栫檪
1.鍖栫檪鍘熷墖锛�
鍩轰簬 LBL 鐢熺墿瀛�(xu茅)鐗规€ч浼间簬 ALL锛岃繎骞翠締(l谩i)鍦�(gu贸)闅涗笂閲囩敤椤炰技 ALL 鐨勫寲鐧傛柟妗堝悗椤憲鏀瑰杽浜嗛爯(y霉)鍚�锛屾瘮杓冨湅(gu贸)闅涚稉(j墨ng)鍏告柟妗�锛屼互 BFM90 鐧傛晥鏈€浣�锛屾湰寤鸿鎻愪緵鐨勬不鐧傛柟妗堟槸 浠� BFM90 鐐哄熀绀�(ch菙)鐨勬敼鑹柟妗�锛屽悇閱�(y墨)闄㈠彲鏍规摎(j霉)鍚勮嚜鎯呮硜閬告搰鎳�(y墨ng)鐢�銆傚厑瑷卞皪(du矛)鎵€閲囩敤鐨勬柟妗堥€�(j矛n)琛屽井瑾�(di脿o)锛屽崁(q奴)鍩熶腑蹇冩垨澶х殑閱�(y墨)鐧備腑 蹇冨彲鍦ㄥ師鍓囦笉璁婄殑鍩虹(ch菙)涓婃彁鍑哄悎鐞嗕慨鏀�銆�
2.鍖栫檪鍓嶆簴(zh菙n)鍌�
(1)纰鸿ê妾㈡煡锛氱洝鍙兘鍦ㄦ渶瀹规槗鍙栨潗鐨勯儴浣嶅彇寰楁(bi膩o)鏈皨 鎵捐叓鐦ょ窗(x矛)鑳烇紝鍖呮嫭锛氶楂�銆佽兏鑵规按銆佹泛琛ㄦ穻宸寸祼(ji茅)銆佺毊鑶氬彲鐤戠槫鐏躲€佹繁閮ㄦ穻宸寸祼(ji茅)绌垮埡鎴栨椿妾�銆佽兏鑵硅厰鐦ょ伓绌垮埡鎴栨墜琛�(sh霉)鍙栨椿 妾€€傜劧鍚庡畬鍠勮叓鐦ょ窗(x矛)鑳炵殑 MICM 鍒嗗瀷;瀹屾垚涓績鐥呯悊鏈�(hu矛)瑷�(鑷冲皯鍏╁涓夌敳閱�(y墨)闄㈢梾鐞嗚€佸斧鏈�(hu矛)瑷�)銆�
(2)寤鸿鑳搁鍙婇珎楠ㄥ叐鍊�(g猫)閮ㄤ綅楠ㄩ珦绌垮埡鎴栨椿妾細鎳�(y墨ng)琛岄 楂撶窗(x矛)鑳炲舰鎱�(t脿i)瀛�(xu茅)銆佹祦寮忕窗(x矛)鑳炲厤鐤〃鍨嬪垎鏋�銆佺窗(x矛)鑳為伜鍌冲(xu茅)鍜屽熀鍥犳鏌�銆�
(3)甯歌(gu墨)妾㈡煡锛氬寘鎷叏琛€绱�(x矛)鑳炲垎鏋�銆佺櫧绱�(x矛)鑳炲垎椤炪€佽倽鑵� 鍔熻兘銆佽娓呬钩閰歌劔姘叾銆佽兏鑵规按鍙婂叾浠栭珨娑茬殑绱�(x矛)鑳炲(xu茅)妾㈡煡绛�銆�
(4)鑵﹁剨娑叉鏌ワ細绱�(x矛)鑳為洟蹇冩硶鎵剧槫绱�(x矛)鑳�銆佹祦寮忔柟娉曟娓�(c猫) 寰噺绱�(x矛)鑳炪€�
(5)褰卞儚瀛�(xu茅)妾㈡煡閫�(j矛n)琛屽垎鏈燂細琛屽彲鐤戞穻宸寸祼(ji茅)鍙婅吂閮� B 瓒�銆� 鍙枒鍙椾镜閮ㄤ綅鐨勯楠肩墖銆丆T 绛夊奖鍍忓(xu茅)妾㈡煡浠ヤ究浜嗚В鐥呰畩鑼冨湇銆� 鎳风枒涓绁炵稉(j墨ng)绯荤当(t菕ng)鐥呰畩鏅�(sh铆)鍙鑵﹀強鑴婇珦纾佸叡鎸�(MRI)妾㈡煡銆� 鏈夋浠剁殑琛� PET/CT 妾㈡煡銆�
(6)鍖栫檪鍓嶆鏌ワ細浜嗚В鑷熷櫒鍔熻兘銆佸厤鐤媭娉併€佺梾姣掓劅鏌�銆� 鍌虫煋鐥呯瓑鐙€娉�銆�
3.甯歌(gu墨)鍖栫檪锛氫互 BFM90 鐐哄熀绀�(ch菙)鐨勬不鐧傛柟妗堝寘鎷� VDLP+CAM 瑾樺皫(d菐o)绶╄В娌荤檪銆�4 鐧傜▼ HD-MTX(鐢叉皑铦跺懁)鎴� 6 鐧傜▼楂樺嵄鏂� 妗堢殑绶╄В鍚庨瀼鍥烘不鐧�銆乂DLD+CAM 寤堕伈寮�(qi谩ng)鍖栨不鐧�銆�6-MP+MTX 鐨勭董鎸佹不鐧傜瓑鐠�(hu谩n)绡€(ji茅)銆�
涓嶅悓浜� ALL锛孡BL 涓厾銆佲叀鏈熸偅鑰呯劇(w煤)寤堕伈 寮�(qi谩ng)鍖栤叀娌荤檪锛屽欢閬插挤(qi谩ng)鍖栤厾娌荤檪鍚庣洿鎺ラ€�(j矛n)鍏ョ董鎸佹不鐧�銆傝垏鍏跺畠椤炲瀷 NHL 涓嶅悓锛孡BL 鍦ㄩ仈(d谩)鍒板畬鍏ㄧ珐瑙e悗锛岄倓闇€閫�(j矛n)琛岀董鎸佹不鐧�锛屽叾鐩殑鏄獦灏�(d菐o)鑵槫绱�(x矛)鑳炲垎鍖�銆佸噵浜★紝閬�(d谩)鍒板竟搴曟竻闄ゆ畼鐣欑梾鐏� 鐨勭洰鐨�?锛熷仐鐓剁⒊s鐐� 24 鍊�(g猫)鏈�銆傛不鐧傞亷(gu貌)绋嬩腑闇€瀹氭湡閫�(j矛n)琛岃⿻(p铆ng)浼�锛屽寘鎷楂撳父瑕�(gu墨)銆侀楂� MRD 鐨勬娓�(c猫)鍙婄槫鐏剁殑瑭�(p铆ng)浼�锛屼簡瑙g珐瑙g媭 鎱�(t脿i)锛屽強鏅�(sh铆)瑾�(di脿o)鏁存不鐧傛柟妗�銆�
4.涓绁炵稉(j墨ng)绯荤当(t菕ng)娴告饯(r霉n)鐨勯爯(y霉)闃插強娌荤檪锛氬師鍓囦笂鍘婚櫎浜嗛”鑵� 鏀剧檪(鍖呮嫭 CNS3 鐥呬汉)锛屼互鍔犲挤(qi谩ng)鍖栫檪銆佸鍔犻灅娉ㄥ姞寮�(qi谩ng) CNS 渚� 鐘殑闋�(y霉)闃插拰娌荤檪銆�
(1)CNS1 鐨勬不鐧傦細鎵€鏈夌梾浜哄潎鎺ュ彈闋�(y霉)闃叉€т笁鑱�(li谩n)闉樻敞鍙� 4 鐧傜▼澶у姂閲� MTX 鍖栫檪锛屽叾涓� T-LBL 5 g/m² ;B-LBL 3 g/m²銆� 鏁村€�(g猫)鍖栫檪閬�(gu貌)绋嬩腑锛孊-LBL 鑷冲皯 IT 20 娆★紝T-LBL 22 娆�銆�
(2)CNS2 鐨勬不鐧傦細鍦ㄨ獦灏�(d菐o)绶╄В VDLP 鏂规涓�(绗� 8銆�22 澶�)澧炲姞 2 娆′笁鑱�(li谩n)闉樻敞锛屽嵆姣忓懆涓€娆�锛屽叡 5 娆°€傚叏绋嬪叡 22锝� 24 娆�銆�
(3)CNS3 鐨勬不鐧傦細鍦ㄨ獦灏�(d菐o)绶╄В VDLP 鏂规涓�(绗� 8銆�22 澶�)澧炲姞 2 娆′笁鑱�(li谩n)闉樻敞锛屽嵆姣忓懆 1 娆�锛屽叡 5 娆�銆傚欢閬插挤(qi谩ng)鍖栤厾 VDLD (绗� 1銆�15 澶�)澧炲姞 2 娆¢灅娉�銆傚叏绋嬪叡 26锝�30 娆�銆�
(4)缍寔娌荤檪鏈熼枔鐨� CNS 娌荤檪锛欳NS2(鍖呮嫭涓鑷ㄨ繎閮ㄤ綅渚电姱鑰�)銆丆NS3 鐥呬汉缍寔娌荤檪鏈熼枔澧炲姞鍏╂闉樻敞銆俆-LBL 缍寔娌荤檪鏈熼枔姣� 4 鍛ㄩ灅娉� 1 娆�锛屽叡 12 娆�;B-LBL 姣� 8 鍛ㄩ灅娉� 1 娆★紝鍏� 8 娆°€� 娉細鎸夊勾榻′笁鑱�(li谩n)闉樻敞鍔戦噺(瑕�(ji脿n)闄勮〃 3)銆�
5.鐫父娴告饯(r霉n)鐨勬不鐧傚師鍓�
(1)鐣�(d膩ng)瑷烘柗鏅�(sh铆)瀛樺湪鐫父鐒�(w煤)鐥涙€ц叓澶х瓑鍏稿瀷鐫父娴告饯(r霉n)鐨勭櫏 鐙€鏅�(sh铆)锛屼笉涓€瀹氬繀闋堣娲绘锛屽彲閫氶亷(gu貌) B 瓒呯⒑瑷�銆傝嫢缍�(j墨ng)閬�(gu貌)瑾樺皫(d菐o)鍙� 闉忓浐娌荤檪鍚庣潽涓歌叓鐗╂秷澶�锛孊 瓒呮鏌ョ潽涓稿(sh铆)璩�(zh矛)鍥炶伈鎭㈠京(f霉)姝e父鍙笉鏀剧檪銆�
(2)鑻ョ稉(j墨ng)閬�(gu貌)瑾樺皫(d菐o)鍙婇瀼鍥烘不鐧傚悗浠嶆湁鎸佺簩(x霉)瀛樺湪鐨勭潽涓歌叓 鐗╋紝闇€琛屾椿妾�銆傜稉(j墨ng)鐥呯悊璀夊(sh铆)浠嶅瓨鍦ㄧ潽涓告蹈娼�(r霉n)鑰�锛屼簬闉忓浐娌荤檪 M 鍚庨渶琛岄洐鍋�(c猫)鐫父鏀剧檪锛屽姂閲忕偤 18-24Gy銆�
(浜�)鍖栫檪鏂规
1.鍒濆瑾樺皫(d菐o)鍖栫檪鏂规锛歏DLP 鏂规+鍏╃檪绋� CAM 鏂规銆�
(1)VDLP 鏂规瑾樺皫(d菐o)娌荤檪锛氶暦(zh菐ng)鏄ユ柊鍫�(VCR)1.5 mg/(m²·d)锛屾瘡鍛� 1 娆�锛屽叡 4 娆�锛� 姣忔鏈€澶х禃灏�(du矛)閲忎笉瓒呴亷(gu貌) 2 mg锛宒8銆乨15銆乨22銆乨29锛屽叡 4 娆�;鏌旂磪闇夌礌(DNR)25 mg/(m²·d)锛岄潨鑴堟淮娉� 6 灏忔檪(sh铆)鍏�锛� 姣忓懆 1 娆�锛宒8銆乨15銆乨22銆乨29锛屽叡 4 娆�; 宸︽棆闁€鍐叞鑳洪叾(L-Asp)5000 u/(m²·d)锛宒8銆乨11銆� d14銆乨17銆乨20銆乨23銆乨26銆乨29锛孮2d锛屽叡 8 娆�锛岃倢鑲夋敞灏�; 鑻� L- ASP 閬�(gu貌)鏁忓彲浜堝煿闁€鍐叾(PEG-ASP)2500 IU/m²锛岃倢鑲� 娉ㄥ皠锛宒9锛宒23锛屽叡 2 鍔�;娼戝凹鏉�(PDN)60 mg/(m²·d)锛宒1锝瀌28锛屽彛鏈�锛岀 29锝� 35 澶╅仦娓涜嚦鍋溿€� PDN 灏�(du矛)浜� 鑵� 鐦� 璨�(f霉) 鑽� 澶� 鐨� 鎮� 鑰� 璧� 濮� 鍔� 閲� [(0.2 锝� 0.5 mg/(kg·d)]闁嬪涓€鍛ㄥ収(n猫i)閫愭几澧炶嚦 60mg/(m²·d)锛屼互鍏嶇櫦(f膩)鐢� 鑵槫婧惰В缍滃悎寰�銆� 鍠伅(li谩n) MTX 闉樻敞锛宒1;涓夎伅(li谩n)闉樻敞(TIT)锛孋NS1锛歞15銆乨33; CNS2銆丆NS3锛歞8銆乨15銆乨22銆乨33銆�
(2)CAM 鏂规锛氳嫢鎮e厭鐒�(w煤)鐧�(f膩)鐔�銆佺劇(w煤)鍤�(y谩n)閲嶆劅鏌�;ANC≥0.5 ×10⁹/L锛孭LT≥50×10⁹/L 鏅�(sh铆)锛屽彲浜� d36 闁嬪绗竴杓� CAM 鏂规銆� 鍏� 2 鍊�(g猫)鐧傜▼ CAM 鏂规锛屽叿楂旇棩鐗╄(ji脿n)涓嬶細 鐠�(hu谩n)纾烽叞鑳�(CTX)1000 mg/(m²·d)锛�1 娆★紝d1锛屾按鍖栧牽鍖� 3 澶�; 缇庡徃閭� 400mg/(m²·娆�)锛屼簬闈滆剤婊存敞 CTX 鐨� 0h銆�4h銆�8h锛� d1; 闃跨硸鑳炶嫹(Ara-C)75 mg/(m²·d) 锛宒3锝瀌6锛宒10锝瀌13, 鍏� 8 澶�; 6-宸板熀鍢屽懁(6-MP)60 mg/(m²·d)锛屽叡 14 澶�銆� 涓夎伅(li谩n)闉樻敞锛宒10銆� 浼戠檪 1-2 鍛�;ANC≥0.5×10⁹/L锛孭LT≥50×10⁹/L;鐒�(w煤)鐧�(f膩) 鐔卞強鍤�(y谩n)閲嶆劅鏌�;闁嬪绗� 2 杓� CAM銆�
2.绶╄В鍚庨瀼鍥烘不鐧�
(1)浣庡嵄绲勫拰涓嵄绲勯瀼鍥烘不鐧� M 鏂规锛� CAM2 绲�(ji茅)鏉熷悗 1-2 鍛紝鐒�(w煤)鐧�(f膩)鐔�銆佺劇(w煤)鍤�(y谩n)閲嶆劅鏌�;鑲岄厫娓呴櫎鐜� 鍊兼甯�;鑲濆姛鑳� ALT/AST≤10 鍊嶆甯镐笂闄愬€�;鑶界磪绱�≤3 鍊� 姝e父涓婇檺鍊�锛孉NC≥0.5×10⁹/L锛孭LT≥50×10⁹/L 鏅�(sh铆)鍙互闁嬪 闉忓浐娌荤檪鏂规 M銆� CAM2 鍚庤璞″洖鍗囧嵆寰�(f霉)鏌ラ绌�锛屽苟琛岀槫鐏惰⿻(p铆ng)浼�锛屾湭瑕�(ji脿n)鏄� 纰烘畼鐣欑梾鐏�锛屽彲浠ラ枊濮� M 鏂规鍖栫檪銆傚叿楂旇棩鐗╁涓嬶細澶у姂閲忕敳姘ㄥ枊鍛�(MTX)3锝�5 g/(m²·d),姣�?j墨)鑺�?1 娆�锛� 鍏� 4 娆T-LBL:5 g/(m²·娆�);B-LBL:3g/(m²·娆�)]锛宒8銆乨22銆� d36銆乨50;鍥涙矮钁夐吀閳�(CF)15 mg/m²锛�6 灏忔檪(sh铆) 1 娆�锛�3锝�8 娆�锛屾牴 鎿�(j霉) MTX 琛€钘ユ績搴︾郸浜堣(di脿o)鏁�銆� 6-MP 25 mg/(m²· d)锛屼笉瓒呴亷(gu貌) 56 澶�锛屾牴鎿�(j霉) WBC 瑾�(di脿o)鏁村姂 閲忋€� 涓夎伅(li谩n)闉樻敞锛歞8銆乨22銆乨36銆乨50銆� 鎳�(y墨ng)鐢ㄥぇ鍔戦噺 MTX 鏈熼枔闇€瑕侀€�(j矛n)琛屾按鍖栥€佸牽鍖�锛屽苟鐩�(ji膩n)娓�(c猫) MTX 琛€钘ユ績搴�銆�45 灏忔檪(sh铆)鏌� MTX 婵冨害灏忎簬 0.25μmol/L 鐐烘甯搞€傝嫢 MTX 楂樹簬姝e父锛屾牴鎿�(j霉)婵冨害瑾�(di脿o)鏁� CF 鐢ㄩ噺鍜屾鏁�(sh霉)(瑕�(ji脿n)闄勮〃 4)銆�
(2)楂樺嵄绲勯瀼鍥烘不鐧傛柟妗堬細 CAM2 鍚庤璞″洖鍗囧嵆寰�(f霉)鏌ラ绌�锛屽苟琛岀槫鐏惰⿻(p铆ng)浼�锛屾寜鐓у嵄闅�(xi菐n)搴﹀垎绲勯€�(j矛n)鍏ラ珮鍗辩祫鐥呬汉鍙互閫�(j矛n)鍏ラ瀼鍥烘不鐧傞珮鍗�(HR)鏂规鍖� 鐧�銆� CAM2 绲�(ji茅)鏉熷悗 1锝�2 鍛�锛岀劇(w煤)鐧�(f膩)鐔便€佺劇(w煤)鍤�(y谩n)閲嶆劅鏌�;鑲岄厫娓呴櫎 鐜囧€兼甯�;鑲濆姛鑳� ALT/AST≤10 鍊嶆甯镐笂闄愬€�;鑶界磪绱�≤3 鍊嶆甯镐笂闄愬€�;ANC≥1×10⁹/L锛孭LT≥100×10⁹/L 鏅�(sh铆)鍙互闁� 濮� HR 鏂规锛岄爢娆″寘鎷� HR-1銆丠R-2銆丠R-3 鏂规銆�
鈶� HR-1 鏂规锛� DXM 20mg/(m²·d)锛屽彛鏈嶆垨闈滆剤鎺ㄦ敞锛宒1锝瀌5; VCR 1.5 mg/m 2/娆�(鏈€澶� 2 mg)锛岄潨鑴堟帹娉�锛宒1锛宒6; HD-MTX 5 g/(m²·娆�)锛岄潨鑴堟淮娉�锛宒1;CF 15 mg/(m²·娆�)锛� 6 灏忔檪(sh铆) 1 娆�锛�3锝�8 娆�锛屾牴鎿�(j霉) MTX 琛€钘ユ績搴﹁(di脿o)鏁�; CTX 200 mg/(m²·娆�)锛�12 灏忔檪(sh铆) 1 娆�锛岄潨鑴堟淮娉紝d2锝� d4锛屽叡 5 娆�锛孒D-MTX 绲�(ji茅)鏉熷悗 7h 闁嬪浜�;缇庡徃閭� 70 mg/(m²·娆�)锛� 浜庨潨鑴堟淮娉� CTX 鐨� 0h銆�4h銆�8h; Ara-c 2000 mg/(m²·娆�)锛� 12 灏忔檪(sh铆) 1 娆�锛宒5锛屽叡 2 娆�; L-ASP 25000 u/(m²·d)闈滆剤婊存敞 2 灏忔檪(sh铆)浠ヤ笂锛宒6銆乨11; 濡傛灉 L-ASP 閬�(gu貌)鏁� PEG-ASP 2500 u/(m²·娆�)锛岃倢鑲夋敞灏�锛宒6; TIT d1銆�
鈶� HR-2 鏂规锛� DXM 20 mg/(m²·d)锛屽彛鏈嶆垨闈滆剤鎺ㄦ敞锛宒1锝瀌5; 闀�(zh菐ng)鏄ュ湴杈�(VDS)3 mg/(m²·娆�)锛岄潨鑴堟帹娉紝d1锛宒6; HD-MTX 5 g/m²/娆�锛岄潨鑴堟淮娉�锛宒1;CF 15mg/(m²·娆�)锛� 6 灏忔檪(sh铆) 1 娆�锛�3锝�8 娆★紝鏍规摎(j霉) MTX 琛€钘ユ績搴﹁(di脿o)鏁�; 鐣扮挵(hu谩n)纾烽叞鑳�(IFO)800 mg/(m²·娆�)锛岄潨鑴堟淮娉紝12 灏� 鏅�(sh铆) 1 娆�锛宒2锝瀌4锛屽叡 5 娆★紝HD-MTX 绲�(ji茅)鏉熷悗 7h 闁嬪浜�;缇庡徃 閳� 300 mg/(m²·娆�)锛岄潨鑴堟淮娉�锛孖FO 0h銆�4h銆�8h; DNR 30 mg/(m²·娆�)锛岄潨鑴堟淮娉�锛宒5; L-ASP 25000 u/(m²·娆�)锛岄潨鑴堟淮娉� 2 灏忔檪(sh铆)浠ヤ笂锛宒6銆乨11;濡傛灉 L-ASP 閬�(gu貌)鏁� PEG-ASP 2500 u /(m²·娆�)锛岃倢鑲夋敞 灏�锛宒6; TIT d1銆�
鈶� HR-3 鏂规锛� DXM 20 mg/(m²· d)锛屽彛鏈嶆垨闈滆剤鎺ㄦ敞锛宒1锝瀌5; Ara-c 2000 mg/(m²·娆�)锛岄潨鑴堟淮娉�锛�12 灏忔檪(sh铆) 1 娆★紝d1锝� d2; 渚濇墭娉婅嫹(VP-16)100 mg/(m²·娆�)锛岄潨鑴堟淮娉�锛�12 灏� 鏅�(sh铆) 1 娆�锛屽叡 5 娆★紝d3锝瀌5; L-ASP 25000 u/(m²·娆�)锛岄潨鑴堟淮娉� 2 灏忔檪(sh铆)浠ヤ笂锛宒6銆� d11;濡傛灉 L-ASP 閬�(gu貌)鏁� PEG-ASP 2500 u/(m²·娆�)锛岃倢鑲夋敞灏勶紝 d6; TIT d5銆�
3 鐧傜▼HR 鏂规鍚庤楠ㄩ珦鍜岀槫鐏惰⿻(p铆ng)浼帮紝CR 鐨勭梾浜轰箣鍚庡啀閲� 寰�(f霉) HR-1銆丠R-2銆丠R-3 鏂规锛屽熀浜� MTX 婵冨害鐩�(ji膩n)娓�(c猫)鐨勫洓姘憠閰� 瑙f晳銆傚鏋滄湁鐦ょ伓閫�(j矛n)灞曟垨鍑虹従(xi脿n)鏂扮櫦(f膩)鐦ょ伓闇€鍑虹祫锛屾敼 鐢ㄤ簩绶氭柟妗堟不鐧�銆�
3.寤堕伈寮�(qi谩ng)鍖� I 娌荤檪
(1)VDLD 鏂规锛氱暥(d膩ng)闉忓浐鏂规绲�(ji茅)鏉熷悗绱� 2 鍛紝鐒�(w煤)鐧�(f膩)鐔�銆佺劇(w煤) 鍤�(y谩n)閲嶆劅鏌�;ANC≥1×10⁹/L锛孭LT≥100×10⁹/L 鏅�(sh铆)鍙枊濮嬫鏂� 妗堝寲鐧�銆傛湰鐧傜▼鍖栫檪鍓嶅京(f霉)鏌ラ楂擄紝骞惰鐦ょ伓瑭�(p铆ng)浼�銆� 鍏烽珨钘ョ墿濡備笅锛� VCR 1.5 mg/(m²· d)锛屾瘡鍛� 1 娆�锛屼綆鍗辩祫鍜屼腑鍗辩祫鍏� 3娆�锛孌1銆�8銆�15;楂樺嵄绲勫叡 4 娆★紝d1銆乨8銆乨15銆乨22锛屾瘡娆℃渶澶х禃灏�(du矛)閲忎笉瓒呴亷(gu貌) 2 mg; DNR 鎴栭樋闇夌礌(ADR)25 mg/(m²· d)锛屾瘡鍛� 1 娆�锛屼綆鍗� 绲勫拰涓嵄绲勫叡 3 娆�锛宒1銆乨8銆乨15;楂樺嵄绲勫叡 4 娆�锛宒1銆乨8銆� d15銆乨22; L-asp 10000 u/(m²·d)锛岃倢鑲夋敞灏勶紝Q2d锛宒1銆乨4銆乨7銆� d10锛屽叡 4 娆�;L-ASP 閬�(gu貌)鏁忓彲浜堝煿闁€鍐叾(PEG-ASP)2500 IU/(m² 娆�)锛岃倢鑲夋敞灏勶紝d4锛屽叡 1 鍔戙€� DXM 8锝�10mg/(m²· d)锛宒1锝瀌7锛宒15锝瀌21; 涓夎伅(li谩n)闉樻敞锛歞1銆乨15(鍍� CNS3 鑰呭鍔� 2 娆� IT)銆�
(2)CAM 鏂规: 浼戠檪 1锝�2 鍛�;ANC≥1×10⁹/L锛孭LT≥100 ×10⁹/L;鐒�(w煤)鐧�(f膩)鐔卞強鍤�(y谩n)閲嶆劅鏌�;鑲岄厫鍊兼甯�;闁嬪 CAM 鏂规鍖栫檪銆傚叿楂旇棩鐗╁涓嬶細 鐠�(hu谩n)纾烽叞鑳�(CTX)1000 mg/(m²· d)锛�1 娆★紝d1锛屾按鍖栧牽 鍖� 3 澶�;缇庡徃閭� 400 mg/(m²·娆�)锛屼簬闈滆剤婊存敞 CTX 鐨� 0h銆�4h銆� 8h锛宒1; 闃跨硸鑳炶嫹(Ara-C)75 mg/(m²· d)锛宒3锝瀌6锛宒10锝瀌13, 鍏� 8 澶�; 6-宸板熀鍢屽懁(6-MP)60 mg/(m²· d)锛屽叡 14 澶�銆� 涓夎伅(li谩n)闉樻敞锛宒3銆備紤鐧傜磩 2 鍛�锛岀劇(w煤)鐧�(f膩)鐔卞強鍤�(y谩n)閲嶆劅鏌�;ANC≥0.2×10⁹/L锛孭LT ≥50×10⁹/L;鍙枊濮嬬董鎸佹不鐧�銆�
4.涓枔缍寔娌荤檪銆備腑鍗辩祫鐥呬汉瀹屾垚寤堕伈寮�(qi谩ng)鍖朓鍚庨€�(j矛n)鍏� 8 鍛ㄤ腑闁撶董鎸佹不鐧�(鍗崇敤 8 鍛� 6-MP+MTX/VD 鏂规锛屽叿楂旀柟妗堢偤锛� 6-MP 50 mg/(m²·d)锛屾寔绾�(x霉)鐫″墠绌鸿吂鍙f湇鍏� 8 鍛�;MTX 20 mg/m²锛� 姣忓懆 1 娆�锛屽叡 8 娆★紝鍙f湇鎴栬倢鑲夋敞灏�锛屾牴鎿�(j霉) WBC 瑾�(di脿o)鏁存柟妗堜腑 6-MP 鐨勫姂閲忋€俈CR 1.5 mg/(m²· d)锛�1 娆★紝d1锛屾瘡娆℃渶澶х禃灏�(du矛)閲忎笉瓒呴亷(gu貌) 2 mg;DXM 6mg/(m²· d)锛宒1锝瀌5銆備笁鑱�(li谩n)闉樻敞锛� d1銆�
5.寤堕伈寮�(qi谩ng)鍖栤叀娌荤檪
(1)VDLD 鏂规锛氫腑鍗辩祫鐥呬汉瀹屾垚涓枔缍寔娌荤檪鍚庡京(f霉)鏌ラ楂擄紝骞惰鐦ょ伓瑭�(p铆ng)浼�锛岃檿浜庡畬鍏ㄧ珐瑙g媭鎱�(t脿i)锛岄€�(j矛n)鍏ュ欢閬插挤(qi谩ng)鍖栤叀娌� 鐧�銆傜暥(d膩ng)鎮e厭鐒�(w煤)鐧�(f膩)鐔便€佺劇(w煤)鍤�(y谩n)閲嶆劅鏌�;ANC≥1×10⁹/L锛孭LT≥100 ×10⁹/L 鏅�(sh铆)鍙枊濮嬫鏂规鍖栫檪銆傚叿楂旇棩鐗╁涓嬶細 VCR 1.5 mg/(m²· d)锛屾瘡鍛� 1 娆�锛屽叡 3 娆★紝d1銆乨8銆乨15锛� 姣忔鏈€澶х禃灏�(du矛)閲忎笉瓒呴亷(gu貌) 2 mg; DNR 鎴栭樋闇夌礌(ADR)25 mg/(m²· d)锛屾瘡鍛� 1 娆�锛屽叡 3 娆★紝d1銆乨8銆乨15; L-asp 10000 u/(m²· d)锛岃倢鑲夋敞灏勶紝Q2d锛宒1銆乨4銆乨7銆� d10锛屽叡 4 娆�;L-ASP 閬�(gu貌)鏁忓彲浜堝煿闁€鍐叾(PEG-ASP)2500 IU/m²锛� 鑲岃倝娉ㄥ皠锛宒4锛屽叡 1 鍔�銆� DXM 8-10mg/(m²· d)锛宒1锝瀌7锛宒15锝瀌21; 涓夎伅(li谩n)闉樻敞锛歞1銆�
(2)CAM 鏂规: 浼戠檪 1锝�2 鍛�;ANC≥1×10⁹/L锛孭LT≥100 ×10⁹/L;鐒�(w煤)鐧�(f膩)鐔卞強鍤�(y谩n)閲嶆劅鏌�;鑲岄厫鍊兼甯�;闁嬪 CAM 鏂规 鍖栫檪銆傚叿楂旇棩鐗╁涓嬶細 鐠�(hu谩n)纾烽叞鑳�(CTX)1000 mg/(m²· d)锛�1 娆�锛宒1锛屾按鍖栧牽 鍖� 3 澶�; 缇庡徃閭� 400dmg/(m²· 娆�)锛屼簬闈滆剤婊存敞 CTX 鐨� 0h銆�4h銆� 8h锛宒1; 闃跨硸鑳炶嫹(Ara-C)75 mg/(m²· d) 锛宒3锝瀌6锛宒10锝瀌13, 鍏� 8 澶�; 6-宸板熀鍢屽懁(6-MP)60 mg/(m²· d) 锛屽叡 14 澶�銆� 涓夎伅(li谩n)闉樻敞锛宒3銆�
6.缍寔娌荤檪鏂规锛氫綆鍗辩祫鍜岄珮鍗辩祫鐥呬汉瀹屾垚寤堕伈寮�(qi谩ng)鍖朓鍚庨€�(j矛n)鍏ョ董鎸佹不鐧傞殠娈点€傚嵆 6-MP+MTX 鏂规鏈熼枔姣� 4 鍛ㄦ彃鍏� VD 鏂规涓€娆�銆� 闁嬪缍寔娌荤檪鏅�(sh铆)琛岀槫鐏惰⿻(p铆ng)浼帮紝缍寔娌荤檪鍓嶅洓鍊�(g猫)寰挵(hu谩n)姣� 8 鍛ㄨ⿻(p铆ng)浼颁竴娆�(鍖呮嫭楠ㄩ珦妾㈡煡)锛屼互鍚庢瘡 8 鍛ㄥ皬瑭�(p铆ng)浼颁竴娆�(绨�(ji菐n) 鍠奖鍍忓(xu茅)妾㈡煡锛岀劇(w煤)楠ㄧ┛銆佽叞绌�)锛屾瘡 16 鍛ㄥぇ瑭�(p铆ng)浼� 1 娆�(鍖呮嫭 鑵﹁剨娑插拰楠ㄩ珦妾㈡煡)銆�
(1)6-MP+MTX 鏂规锛� 6-MP 50 mg/(m²· d)锛屾寔绾�(x霉)鐫″墠绌鸿吂鍙f湇; MTX 20 mg/m²锛屾瘡鍛� 1 娆�锛屽彛鏈嶆垨鑲岃倝娉ㄥ皠 鏍规摎(j霉) WBC 瑾�(di脿o)鏁存柟妗堜腑鐨勮棩鐗╁姂閲�銆�
(2)VD 鏂规锛� VCR 1.5 mg/(m²·d)锛�1 娆�锛屾瘡娆℃渶澶х禃灏�(du矛)閲忎笉瓒呴亷(gu貌) 2 mg; DXM 6锝�8 mg/(m²· d)锛宒1锝瀌5銆� 绺芥不鐧傜檪绋嬶細 绱� 2.5 骞淬€�
(涓�)LBL 寰�(f霉)鐧�(f膩)鍚庣殑娌荤檪绛栫暐
澶х磩 10%锝�20%閫�(j矛n)灞曟湡 LBL 灞洠娌绘垨寰�(f霉)鐧�(f膩)鐥呬緥銆傜珐瑙e悗涓€ 鏃﹀京(f霉)鐧�(f膩)锛屽線寰€鐥呮儏妤靛叾鍏囬毆(xi菐n)锛岃繀閫熷叏韬鑷熷櫒杞�(zhu菐n)绉�锛屽啀娆$珐 瑙e洶闆�锛岄爯(y霉)鍚庢サ宸�
鐢熷瓨鐜囧儏鐐� 10%锝�30%銆傝(b菙)鏁戞不鐧備富瑕佸寘鎷啀娆¤獦灏�(d菐o)鍜岄€犺骞茬窗(x矛)鑳炴敮鎸佺殑寮�(qi谩ng)鍖� 娌荤檪銆傝(b菙)鏁戠殑鐩(bi膩o)鏄洝蹇仈(d谩)鍒扮┅(w臎n)瀹氱殑浜屾绶╄В(second complete remission 锛� CR2 )锛� 鐩� 鏃� 琛� 閫� 琛€ 骞� 绱�(x矛) 鑳� 绉� 妞� (haematopoietic stem cell transplant锛孒SCT)銆�
鎸芥晳鍖栫檪甯哥敤鐨勪簩绶氭不鐧傜殑绱�(x矛)鑳炴瘨椤炶棩鐗╂湁鐣扮挵(hu谩n)纾烽叞鑳恒€佸幓鐢叉哀鏌� 绱呴湁绱�銆佸崱閴戠瓑銆傜従(xi脿n)鏈夋暩(sh霉)鎿�(j霉)琛ㄦ槑寰�(f霉)鐧�(f膩) LBL 鏈稉(j墨ng) HSCT 骞句箮鐒�(w煤) 娌绘剤鐨勬(j墨)鏈�(hu矛)锛岃€岀浉姣旇嚜楂� HSCT(autologous HSCT锛宎uto-HSCT)锛� 鐣板熀鍥� HSCT(allogenic-HSCT锛宎llo-HSCT)闆栨湁杓冮珮鐨勬不 鐧傜浉闂�(gu膩n)姝讳骸锛屼絾鍏锋湁鏇撮珮鐨勭劇(w煤)鐥呯敓瀛樼巼锛宎uto-HSCT 寰�(f霉)鐧�(f膩)鐜� 鏄庨’楂樹簬 allo-HSCT銆� 1.T-LBL 鎸芥晳娌荤檪
(1)濂堟媺婵�(Nelarabine锛孉rranon)锛氭槸鑴哀槌ヨ嫹椤� 浼肩墿 9-b-D-闃跨硸鍛嬪杻绯栭偿鍢屽懁(ara-G)鐨勫墠楂旇棩鐗�锛屽皪(du矛) T 娣嬪反姣嶇窗(x矛)鑳炵殑 DNA 鍚堟垚鍏锋湁閬告搰鎬ф姂鍒朵綔鐢ㄨ€岃嚧绱�(x矛)鑳炴浜�銆� 鏄竴绋湁鏁堢殑 T 绱�(x矛)鑳炵壒鐣版€х窗(x矛)鑳炴瘨钘ョ墿锛岀敤浜庢不鐧傞洠娌�銆佸京(f霉)鐧�(f膩)鐨� T-LBL/ALL锛屽畠鍙柈钘ユ不鐧傛垨鑸囧叾浠栫窗(x矛)鑳炴瘨钘ョ墿鑱�(li谩n)鐢�銆� 鍏掔鎳�(y墨ng)鐢ㄥ姂閲� 650 mg/(m²· d) ×5 d锛�21d 涓€鐧傜▼锛屸厾/鈪� 鏈熷厭绔ュ強鎴愪汉鐮旂┒锛屽柈钘ュ弽鎳�(y墨ng)鐜� 14%锝�55%銆傚父瑕�(ji脿n)鐨勫壇浣滅敤鐐哄姂閲忎緷璩村瀷绁炵稉(j墨ng)姣掓€�锛岃〃鐝�(xi脿n) 2 绱�(j铆)鎴� 3 绱�(j铆)鎰熻(ju茅)銆侀亱(y霉n)鍕�(d貌ng)绁炵稉(j墨ng)鐥呰畩 鍜岃倢鑲夐楠肩柤鐥�锛屾湁鐨勭梾浜鸿〃鐝�(xi脿n)闋棝銆佸棞鐫�銆佹劅瑕�(ju茅)閬查垗銆佺櫜鐧囩櫦(f膩)浣�銆佸叡婵�(j矛)澶辫(di脿o)绛�锛屽鐐哄彲閫嗘€х殑銆�
鍏朵粬鍓綔鐢ㄧ偤琛€娑叉瘨 鎬�銆佹儭蹇冨様鍚愮瓑娑堝寲閬撳弽鎳�(y墨ng)銆� 濂堟媺婵辨不鐧傚京(f霉)鐧�(f膩)/闆f不 T-ALL/LBL 鏈€甯哥敤鐨勮伅(li谩n)鍚堝寲鐧傛槸 鐠�(hu谩n)纾烽叞鑳哄姞渚濇墭娉婅嫹锛孡eah 绛夊牨(b脿o)閬� 5/7 CR锛屽叾涓� 4 渚嬪緱浠� 閫�(j矛n)琛� HSCT锛屼絾鍍� 1 渚嬪瓨娲�锛屽叾浠栫梾浜烘浜庣柧鐥呴€�(j矛n)灞曞拰娌荤檪鐩� 闂�(gu膩n)鍚堝苟鐧�銆傞櫎浜嗚伅(li谩n)鍚堝寲鐧�锛孠 Burley 鍫�(b脿o)閬� 1 渚嬬Щ妞嶅悗寰�(f霉)鐧�(f膩)鐨� T-ALL 閲囩敤濂堟媺婵卞姞渚涜€呯窗(x矛)鑳炲洖杓搁仈(d谩)鍒版寔绾�(x霉) CR銆侫nnalisa 绛� 鎻愬嚭鍙互鏍规摎(j霉)鐥呬汉鍩哄洜琛ㄩ仈(d谩)璀�锛屾妸濂堟媺婵遍伕鎿囨€у湴鑱�(li谩n)鍚� PI3K銆� Bcl2銆丮EK 鎶戝埗鍔�锛屼互澧炲姞鍏剁檪鏁�銆�
(2)閬�(d谩)闆锋湪鍠姉(Daratumumab)锛欳D38 浜烘簮鍖栧柈鎶�锛� CD38鎶楀師鍦ㄥ寲鐧傚墠鍚庢垨鍦ㄥ京(f霉)鐧�(f膩)鍚嶵-ALL/LBL绱�(x矛)鑳炰笂琛ㄩ仈(d谩)绌�(w臎n)瀹�锛� 鍙兘鎴愮偤鑹ソ鐨勬不鐧傞澏榛�(di菐n)銆傚苟鍙綔鐐� CAR-T 绱�(x矛)鑳炴不鐧傜殑闈堕粸(di菐n)锛岀洰鍓嶅潎鍦ㄦ帰绱腑銆�
(3)鍏朵粬锛氬 PI3K 鎶戝埗鍔戙€乵TOR 鎶戝埗鍔戞垨 PI3K-mTOR 闆欓噸鎶戝埗鍔戣伅(li谩n)鍚堢硸鐨唱(zh矛)婵€绱犵敤浜庨珮鍗� PTEN 缂哄け鐨勭梾浜�;榄� 绱㈠埄鏇垮凹(ruxolitinib)锛欽AK/STAT 閫氳矾鎶戝埗鍔�锛岃兘澶犲厠鏈� 绯栫毊璩�(zh矛)婵€绱犺€愯棩绛夊潎鍦ㄧ爺绌朵腑銆�
2.B-LBL 鎸芥晳娌荤檪
(1)閰皑閰告縺閰舵姂鍒跺姂(TKI)锛氬彲鐢ㄤ簬娌荤檪鍒濇不鍜屽京(f霉)鐧�(f膩)鐨� Ph + ALL / LBL锛岄€氶亷(gu貌)闈跺悜浣滅敤浜� BCR/ABL 铻嶅悎鍩哄洜寰炶€岀櫦(f膩)鎻檪鏁�銆備紛棣浛灏煎柈钘ユ湁鏁堢巼閬�(d谩) 70%锛岃伅(li谩n)鍚堝寲鐧� CR 鐜囧彲閬�(d谩) 90%銆傜浜屼唬閬�(d谩)娌欐浛灏煎拰灏肩緟鏇垮凹鍙敤浜庡皪(du矛)浼婇Μ鏇垮凹鑰愯棩鐨� 鐥呬汉锛屽叿鏈夐€忛亷(gu貌)琛€鑵﹀睆闅滅殑鐗规€э紝鐧傛晥鍎�(y艒u)浜庝紛棣浛灏�銆�
(2)CD19 鐗圭暟鎬у祵鍚堟姉鍘熷彈楂� T 绱�(x矛)鑳�(CAR T 绱�(x矛)鑳�)锛� 娌荤檪寰�(f霉)鐧�(f膩)/闆f不 B-ALL/LBL CR 鐜囬仈(d谩)鍒� 70%锝�92%锛岀檪鏁堥’钁楋紝 绶╄В鍚庡彲姗嬫帴绉绘锛孋AR-T 绱�(x矛)鑳炴不鐧傜殑姣掓€х偤绱�(x矛)鑳炲洜瀛愰噵鏀剧稖鍚堝緛鍜岀缍�(j墨ng)姣掓€�銆�
(3)鍏朵粬濡傞洐鐗圭暟鎬ф姉楂�(Blinatumomab)銆佺〖鏇夸綈绫�(Bortezomib)绛�锛屾不鐧傚京(f霉)鐧�(f膩)/闆f不ALL/LBL鍧囨湁涓€瀹氱檪鏁�銆�
(鍥�)閫犺骞茬窗(x矛)鑳炵Щ妞�
1.灏�(du矛)鍖栫檪閬�(gu貌)绋嬩腑澶氬€�(g猫)瑭�(p铆ng)浼伴粸(di菐n)椤ず灏�(du矛)鍖栫檪涓嶆晱鎰�锛屼腑鏈熻⿻(p铆ng) 浼� 鏈� 鏄� 纰� 娈� 鐣欑梾 鐏� ; 鎴� 瀛� 鍦� 闋�(y霉) 鍚� 涓� 鑹� 鍩� 鍥� 锛� 鍏� 鏈� t(9;22)/BCR-ABL1銆丮LL 閲嶆帓绛�锛屽彲鎸夐珮鍗辩祫鏂规鍖栫檪锛屽畬 鎴愪笁鐧傜▼楂樺嵄鏂规鍖呮嫭 HR-1銆丠R-2銆丠R-3 鍚庤鐣板熀鍥犻€犺 骞茬窗(x矛)鑳炵Щ妞�銆�
2.灏�(du矛)浜庡師鐧�(f膩)鐦ょ伓寰�(f霉)鐧�(f膩)鎴栭楂撳京(f霉)鐧�(f膩)鎴栨不鐧備腑閫�(j矛n)灞曠殑鐥呬汉 閫氶亷(gu貌)鎸芥晳娌荤檪鑳藉閬�(d谩)鍒板畬鍏ㄧ珐瑙f垨閮ㄥ垎绶╄В鐛插緱绉绘姗�(j墨)鏈�(hu矛)鑰�锛岄渶鐩″揩琛岀暟鍩哄洜閫犺骞茬窗(x矛)鑳炵Щ妞嶃€�
鍏�銆佸悎骞剁櫏鐨勮檿鐞�
(涓€)灏�(du矛)瀛樺湪涓婅厰闈滆剤澹撹揩缍滃悎寰�銆佹埃閬撴闃荤殑鐥呬汉鐨� 铏曠悊鍘熷墖 澶х磩 10%T-LBL 鍙兘鍑虹従(xi脿n)鍤�(y谩n)閲嶇殑姘i亾姊楅樆(浼存垨涓嶄即涓婅厰闈滆剤澹撹揩缍滃悎寰�)锛岀偤鐪熸鑵槫鎬ョ櫏銆�
1.灏�(du矛)姝ら鐥呬汉灏氭湭缍�(j墨ng)鐥呯悊纰鸿ê鑰�锛岀蹇屾噳(y墨ng)鐢ㄥ叏韬夯閱夋墜琛�(sh霉)娲绘銆�
2.鍙厛浜堝皬鍖栫檪(濡傛綉灏兼澗 60 mg/m 2鍙f湇鎴� VP 鏂规) 绶╄В鍛煎惛鍥伴洠锛屼簬鐢ㄨ棩鍚� 24锝�48 灏忔檪(sh铆)鍏�(n猫i)鐧ョ媭鎺у埗鍚庣洝鏃╄ 鐥呯悊妾㈡煡锛屽苟鎳�(y墨ng)閬告搰渚佃ゲ鎬ф渶灏忕殑鎿嶄綔纰鸿ê锛屽彲浠ヨ€冩叜鍙栨潗 闋嗗簭?y脿n)妞嬫睏鎾�?銆佽兏鑵规按銆佹泛琛ㄦ穻宸寸祼(ji茅)銆佺毊鑶氭垨鍏朵粬娣鸿〃鍙枒鐦ょ伓銆佹繁閮ㄦ穻宸寸祼(ji茅)鎴栬叓鐗╃┛鍒烘椿妾�銆佺槫鐏舵墜琛�(sh霉)鍙栨椿妾�銆傚寲鐧� 鍓嶉渶绨界讲鏈⒑瑷哄寲鐧傚崝(xi茅)璀版浉锛屽憡鐭ュ闀�(zh菐ng)鍖栫檪鍙兘褰遍熆鐥呯悊绲�(ji茅) 鏋滆€岀劇(w煤)娉曠⒑瑷�銆�
3.灏�(du矛)浼存湁涓婅厰闈滆剤澹撹揩缍滃悎寰佺殑鐥呬汉鎳�(y墨ng)閬垮厤涓婅偄杓告恫銆�
(浜�)鑵槫婧惰В缍滃悎寰佺殑铏曠悊 瀛樺湪楂樿叓鐦よ矤(f霉)鑽风殑銆佸寲鐧傚墠宸叉湁楂樺翱閰歌鐧�銆佽厧鍔熻兘鎼� 瀹�銆佸皯灏挎按鑵殑鐥呬汉鐐虹櫦(f膩)鐢熻叓鐦ゆ憾瑙g稖鍚堝緛鐨勯珮鍗变汉缇�锛岄渶閲囧彇绌嶆サ鐨勯爯(y霉)闃插拰娌荤檪鎺柦銆�
1.鍤�(y谩n)瀵嗙洠(ji膩n)娓�(c猫)鍚勯爡(xi脿ng)鐢熷懡楂斿緛銆佸嚭鍏ラ噺銆侀珨閲嶃€佽闆昏В璩�(zh矛)銆� 鑲濊厧鍔熻兘銆�
2.鎳�(y墨ng)鐢ㄥ垾鍢岄唶娓涘皯灏块吀鐢熸垚锛� 10mg/(kg· d)[300mg/(m²· d)]锛屽垎2锝�3娆″彛鏈嶃€傚繀瑕佹檪(sh铆)鎳�(y墨ng) 鐢ㄥ翱閰告哀鍖栭叾0.1锝�0.15 mg/(kg· 娆�)鍙繀閫熼檷浣庡翱閰告按骞�銆�
3.绌嶆サ姘村寲鍒╁翱锛�3000 ml/(m² d)[125 ml/(m²· h)]锛� 鐢�1/4锝�1/3寮垫恫楂�锛屼竴鑸笉鐢ㄥ惈閴€娑�銆傜郸浜堥€熷翱0.5锝�1 mg/(kg·娆�)锛孮12h鍒╁翱锛屼繚璀夊嚭鍏ュ钩琛★紝缍寔灏块噺澶х磩鍦� 125 ml/(m²· h)銆傝(b菙)鍏呰冻澶犳恫楂�锛屽彲涓嶅繀鍫垮寲锛屼互闃插牽鍖栨浠朵笅纾烽吀楣藉拰榛冨槍鍛ゅ湪鑵庡皬绠℃矇绌嶉€�(j矛n)涓€姝ュ姞閲嶈厧鎼嶅偡銆�
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涓�銆佹不鐧備腑鍏朵粬娉ㄦ剰浜嬮爡(xi脿ng)
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