涓€銆佹杩�

闁撹畩鎬уぇ绱�(x矛)鑳炴穻宸寸槫(anaplasticlargecelllymphoma锛孉LCL)鏄疶绱�(x矛)鑳炰締婧愮殑鎯℃€ф穻宸寸槫锛岃叓鐦ょ窗(x矛)鑳炲叿鏈夎睈瀵岀殑銆佸褰㈡€х殑鑳炴伎鍜岄Μ韫勫舰鐨勭窗(x矛)鑳炴牳锛岃〃閬�(d谩)CD30鎶楀師銆傛湰鐥呭緢澶т竴閮ㄥ垎鎮ｈ€呰垏t(2;5)(p23锛宷35)鐩搁棞(gu膩n)锛屾槗浣嶅皫(d菐o)鑷寸殑ALK鍩哄洜鐣板父琛ㄩ仈(d谩)鑸囪叓鐦ょ殑鐧�(f膩)鐢�銆佺敓鐗╁(xu茅)鐗规€т互鍙婃偅鑰呯殑闋�(y霉)鍚庢湁鏄庣⒑鐨勭浉闂�(gu膩n)鎬�銆�

鍥犳2008骞寸殑WHO鍒嗛锛岄€�(j矛n)涓€姝ユ牴鎿�(j霉)鏄惁瀛樺湪ALK鍩哄洜鐨勭暟甯歌〃閬�(d谩)锛屽皣ALCL鍒嗙偤鍏╁€嬩笉鍚岀殑鐤剧梾锛欰LK+ALCL鍜孉LK–ALCL銆侫LCL鍗犳垚浜洪潪闇嶅閲戞穻宸寸槫鐨�1%锝�2%锛屽厭绔ユ穻宸寸槫鐨�10%锝�15%锛屽苟浠ョ敺鎬у崰澶氭暩(sh霉)(鐢凤細濂�=1.5锛�1)銆傚湪鍏掔鍜岄潚灏戝勾鎮ｈ€呬腑瓒呴亷90%鐨勬偅鑰呯偤ALK+ALCL锛岃€屾垚浜烘偅鑰呬腑鍍�40%锝�50%鐐篈LK+ALCL銆�

浜屻€侀仼鐢ㄨ寖鍦�

缍�(j墨ng)鐥呯悊銆佸厤鐤祫鍖�銆佸熀鍥犳娓⒑瑷虹偤ALK闄芥€х郴绲�(t菕ng)鎬ч枔璁婃€уぇ绱�(x矛)鑳炴穻宸寸槫鐨勬偅鑰�銆備笉鍖呮嫭ALK闄版€х殑闁撹畩鎬уぇ绱�(x矛)鑳炴穻宸寸槫鍜岀毊鑶氬瀷闁撹畩鎬уぇ绱�(x矛)鑳炴穻宸寸槫銆�

涓�銆佽ê鏂�

(涓€)鑷ㄥ簥琛ㄧ従(xi脿n)

甯告湁鐧�(f膩)鐔辩瓑B绲勭櫏鐙€锛屼镜鐘穻宸寸祼(ji茅)锛屾サ鏄撶櫦(f膩)鐢熸穻宸寸祼(ji茅)澶栦镜鐘�锛屼娇鑷ㄥ簥琛ㄧ従(xi脿n)澶氱ó澶氭ǎ銆�

1. 涓€鑸櫏鐙€锛�

鎮ｈ€呯稉(j墨ng)甯镐即闅˙绲勭櫏鐙€锛岀壒鍒ユ槸楂樼啽銆傚ぇ閮ㄥ垎鎮ｈ€�(70%)鍦ㄧ⒑瑷烘檪宸查仈(d谩)鈪綖鈪ｆ湡銆�

2. 娣嬪反绲�(ji茅)渚电姱锛�

宸寸祼(ji茅)鑵ぇ绱勫湪90%鐨勬偅鑰呬腑鍑虹従(xi脿n)锛屽鍛ㄦ穻宸寸祼(ji茅)鏈€甯稿彈绱�锛屽叾娆℃槸鑵硅啘鍚庢穻宸寸祼(ji茅)鍜岀副闅旇叓鐗�锛屾穻宸寸祼(ji茅)鎾暎鐨勬柟寮忔棦鍙偤閫ｇ簩(x霉)鐨�锛屼篃鍙笉閫ｇ簩(x霉)锛屼镜鐘殑娣嬪反绲�(ji茅)鍙偤鐒＄棝鎬ц叓澶�锛屼篃鍙即鏈夌磪鑵啽鐥涖€�

3. 绲�(ji茅)澶栦镜鐘細

ALK+ALCL鎮ｈ€呰純鍏朵粬浜炲瀷鐨凬HL鎮ｈ€呮洿澶�(60%鎮ｈ€�)鏇存棭鍦板嚭鐝�(xi脿n)鍖呮嫭鐨啔銆侀銆佽粺绲勭箶銆佽偤鍦ㄥ収(n猫i)鐨勭祼(ji茅)澶栦镜鐘�銆傜祼(ji茅)澶栦镜鐘殑鑷ㄥ簥琛ㄧ従(xi脿n)澶氱ó澶氭ǎ銆�

(1)杌熺祫绻斿拰鐨啔锛氭槸鏈€甯稿嚭鐝�(xi脿n)鐨勭祼(ji茅)澶栧彈绱儴浣�锛屽彲鐐哄鍊嬫垨鍠€嬬槫鐏�锛岃〃鐝�(xi脿n)鐐虹毊涓嬬祼(ji茅)绡€(ji茅)銆佸ぇ鐨勬桨鐦�銆佸鐧�(f膩)鎴栧綄婕殑涓樼柟妯ｇ磪榛冭壊鐨悕绛�銆�

(2)楠細楠ㄤ镜鐘篃寰堝父瑕�锛屽緸灏忕殑婧堕鎬ф悕瀹冲埌楠ㄧ槫妯ｇ殑澶х槫鐏跺潎鍙銆�

(3)鑲猴細鑲洪儴鐥呰畩鍙偤绲�(ji茅)绡€(ji茅)妯ｆ垨娴告饯妯�锛屽叾涓磩20%鐨勬偅鑰呬即鏈夋儭鎬ф徊鍑�銆�

(4)楠ㄩ珦锛氶楂撲镜鐘殑鎮ｈ€呬笉瓒�15%锛岄楂撴椿妾㈡瘮楠ㄩ珦绌垮埡闄芥€х巼鏇撮珮銆傜敤PCR鐨勬柟娉曟娓琋PM-ALK鍙互鍦�50%鎮ｈ€呯殑楠ㄩ珦涓娓埌鑵槫鐨勫井灏忔挱鏁�銆�

(5)涓绁炵稉(j墨ng)绯荤当(t菕ng)锛氭偅鑰呭湪瑷烘柗鏅傚緢灏戝嚭鐝�(xi脿n)涓绁炵稉(j墨ng)绯荤当(t菕ng)(CNS)渚电姱锛屽瑕嬩簬鏅氭湡鎮ｈ€呫€侰NS鐤剧梾鍙互琛ㄧ従(xi脿n)鐐鸿叇鑶滀镜鐘�(閫氶亷鍦ㄨ叇鑴婃恫涓壘鍒癆LCL绱�(x矛)鑳炵⒑瑷�)鍜�(鎴�)鍑虹従(xi脿n)椤卞収(n猫i)鑵墿銆�

(6)鍏跺畠閮ㄤ綅锛氱潽涓稿彈绱湪ALCL妤电偤缃曡锛岃儼鑵�锛岃厧鑷�锛岃倽鑷�锛岃吀閬撶殑渚电姱涔熻純灏戣銆�

4.楂樼窗(x矛)鑳炲洜瀛愯鐧ワ細

鏈梾姣斿叾浠栭鍨嬬殑娣嬪反鐦ゆ洿鏄撳嚭鐝�(xi脿n)楂樼窗(x矛)鑳炲洜瀛愯鐧�(hypercytokinemia)锛岀敋鑷冲紩鐧�(f膩)鐐庢€у洜瀛愰ⅷ(f膿ng)鏆村拰鍣绱�(x矛)鑳炵稖鍚堝緛(hemophagocyticsyndrome锛孒PS)銆�

(浜�)瀵﹂瀹ゆ鏌�

1.鐥呯悊

(1)鐥呯悊鐗归粸(di菐n)鍜屽垎鍨嬶細

鏈梾鐐鸿叓鐦ょ窗(x矛)鑳炲挤(qi谩ng)琛ㄩ仈(d谩)CD30锛屽湪绔囧収(n猫i)鐢熼暦鐨勫ぇ绱�(x矛)鑳炶叓鐦�銆傞€欎簺鑵槫绱�(x矛)鑳炲ぇ涓斿叿鏈夊褰㈡€�锛岄€氬父鏈夎睈瀵岀殑鑳炴伎锛屾湁鍋忓績鐨�銆侀Μ韫勫瀷鎴栬厧鍨嬬窗(x矛)鑳炴牳锛屾牳鏃佺稉(j墨ng)?锛熸ⅱ濞涗汉宓濊─^(q奴)鍩燂紝閫欎簺鑵槫绱�(x矛)鑳炶绋辩偤妯�(bi膩o)蹇楁€х窗(x矛)鑳�(hallmarkcells)锛屽嚭鐝�(xi脿n)浜庢墍鏈堿LCL鐨勫舰鎱�(t脿i)瀛�(xu茅)浜炲瀷涓�銆�

鐩＄鍏稿瀷鐨勬(bi膩o)蹇楁€х窗(x矛)鑳炵偤澶х窗(x矛)鑳烇紝浣嗕篃鍙鍒板舰鎱�(t脿i)鐩镐技鐨勫皬绱�(x矛)鑳炪€侫LK+ALCL鍏锋湁寤ｆ硾鐨勫舰鎱�(t脿i)璀滐紝鍚勪簽鍨嬪潎浼存湁绋嬪害涓嶅悓鐨勭値鎬ц儗鏅�銆�

2008骞碬HO鍒嗛涓弿杩颁簡浜斿€嬩富瑕佺殑褰㈡厠(t脿i)瀛�(xu茅)浜炲瀷锛�

鈶犳櫘閫氬瀷(commonpattern)锛氱磩鍗�60%銆傜敱鍓嶈堪鍏锋湁妯�(bi膩o)蹇楁€х窗(x矛)鑳炵壒榛�(di菐n)鐨勫褰㈡€уぇ鑵槫绱�(x矛)鑳炵偤涓荤祫鎴愩€傜暥(d膩ng)娣嬪反绲�(ji茅)绲�(ji茅)妲�(g貌u)娌掓湁瀹屽叏鐮村鏅�锛岃叓鐦ょ壒寰佹€у湴鍦ㄧ珖鍏�(n猫i)鐢熼暦锛屼篃鍙镜鐘粯鐨唱(zh矛)鍗€(q奴)銆傞浼肩檶妯ｇ殑绮橀檮妯ｇ敓闀锋柟寮忓緢鍍忚綁(zhu菐n)绉荤槫銆�

鈶℃穻宸寸祫绻旂窗(x矛)鑳炲瀷(lymphohistiocyticpattern锛孡H)-ALCL锛氱磩鍗�10%銆傚叾鐗归粸(di菐n)鏄叓鐦ょ窗(x矛)鑳炵稉(j墨ng)甯告瘮鏅€氬瀷鐨勫皬锛屽彚闆嗕簬琛€绠″懆鍦�銆傝叓鐦ょ窗(x矛)鑳炰腑娣锋湁澶ч噺鍙嶆噳(y墨ng)鎬х殑绲勭箶绱�(x矛)鑳�锛岀壒榛�(di菐n)鐐烘湁绱�(x矛)椤嗙矑鐨勫棞閰告€ц優婕�锛屽拰灏�銆佸湏銆佸舰鎱�(t脿i)涓€鑷寸殑绱�(x矛)鑳炴牳銆備即鏈夋暩(sh霉)閲忎笉绛夌殑婕跨窗(x矛)鑳�銆�

绲勭箶绱�(x矛)鑳炲彲浠ラ伄钃嬭叓鐦ょ窗(x矛)鑳�锛岄倓鍙鍒扮祫绻旂窗(x矛)鑳炲悶鍣磪绱�(x矛)鑳炵殑鐝�(xi脿n)璞★紝鏁呮槗瑾よê鐐哄弽鎳�(y墨ng)鎬х祫绻旂窗(x矛)鑳炵梾銆傝ê鏂风殑闂�(gu膩n)閸垫槸鍏嶇柅绲勫寲鏌撹壊浣跨敤CD30鍜孉LK鎶楅珨锛屽彲浠ュ嚫椤暎鍦ㄤ簬绲勭箶绱�(x矛)鑳炰腑鐨�銆佹槗闆嗕腑浜庤绠″懆鍦嶇殑鑵槫绱�(x矛)鑳�銆�

鈶㈠皬绱�(x矛)鑳炲瀷(smallcellpattern锛孲C)-ALCL锛氱磩鍗�5%锝�10%銆傚皬鍒颁腑绛夊ぇ灏忕殑鑵槫绱�(x矛)鑳炲崰绲曞ぇ澶氭暩(sh霉)锛屾湁涓嶈(gu墨)鍓囩殑绱�(x矛)鑳炴牳鍜岃睈瀵岀殑鑳炴伎銆傛(bi膩o)蹇楁€ц叓鐦ょ窗(x矛)鑳炴暎鍦ㄥ叾涓緢闆ｆ娓埌銆�

浣嗚绠″懆鍦嶅垎甯冪殑妯�(bi膩o)蹇楁€х窗(x矛)鑳炴湁鍔╀簬瑷烘柗銆傚皬鐨勮叓鐦ょ窗(x矛)鑳濩D30鏌撹壊鍙儏鐐哄急闄芥€э紝ALK鏌撹壊甯稿儏闄愪簬鑵槫绱�(x矛)鑳炵殑绱�(x矛)鑳炴牳闄芥€�銆傞€欑祫鐥呬汉鏄撹垏澶栧懆T绱�(x矛)鑳炴穻宸寸槫(闈炵壒鎸�)娣锋穯銆�

鈶ｉ湇濂囬噾妯ｅ瀷(Hodgkin-likepattern锛孒L)-ALCL锛氱磩鍗�3%銆傚叾褰㈡厠(t脿i)鐗归粸(di菐n)寰堣薄绲�(ji茅)绡€(ji茅)纭寲鍨嬬稉(j墨ng)鍏搁湇濂囬噾娣嬪反鐦�銆侰D15鐨勮〃閬�(d谩)闈炲父缃曡锛屼絾鏈塒AX5鐣板父琛ㄩ仈(d谩)鏅傚皪HL-ALCL鐨勮ê鏂锋槸鍊嬫寫鎴�(zh脿n)銆�

鈶ゆ贩鍚堝瀷(compositepattern)锛氱磩鍗�15%銆傚湪鍠竴娣嬪反绲�(ji茅)涓彲瑕嬪埌涓嶅彧涓€绋舰鎱�(t脿i)椤炲瀷銆傛敞锛氳兘澶犺瓨鍒ヨ畩鐣扮殑鐥呯悊浜炲瀷闈炲父閲嶈锛屽惁鍓囨湁瑾よê鐨勫彲鑳�銆傚京(f霉)鐧�(f膩)鎮ｈ€呯殑褰㈡厠(t脿i)瀛�(xu茅)鐗瑰緛涔熷彲浠ヨ垏鍘熺櫦(f膩)鏅備笉鍚�銆�

(2)鍏嶇柅琛ㄥ瀷:

鑵槫绱�(x矛)鑳炵殑绱�(x矛)鑳炶啘鍜岄珮鐖惧熀楂斿崁(q奴)鍩烠D30鏌撹壊闄芥€�锛屽ぇ鐨勮叓鐦ょ窗(x矛)鑳炴煋鑹叉渶寮�(qi谩ng)锛屽皬鐨勭窗(x矛)鑳炲彲鐐哄急闄芥€�銆傝叓鐦ょ窗(x矛)鑳炶〃閬�(d谩)涓€鍊嬫垨澶氬€婽绱�(x矛)鑳炴姉鍘燂紝閭勬湁閮ㄥ垎鎮ｈ€呯敱浜庝笩澶变簡T绱�(x矛)鑳炴姉鍘熻€屾垚鐐�“瑁哥窗(x矛)鑳�(nullcell)”琛ㄥ瀷锛屼絾鍦ㄥ熀鍥犳按骞冲彲浠ユ壘鍒板叾渚嗘簮浜嶵绱�(x矛)鑳炵郴鐨勪緷鎿�(j霉)銆�

鐢变簬T绱�(x矛)鑳炶〃鍨嬭垏瑁哥窗(x矛)鑳炶〃鍨嬬浉姣旇嚚搴婄壒寰佺劇鏄庨’宸垾锛屾晠灏囧叐鑰呰鐐哄悓涓€鐤剧梾銆侰D3鍦ㄨ秴閬�75%鐨勭梾渚嬩腑鐐洪櫚鎬�锛孋D2锛孋D5锛孋D4鍦�70%鐨勬偅鑰呬腑鐐洪櫧鎬�锛孋D8甯哥偤闄版€�锛�2/3鎮ｈ€匔D43闄芥€�銆傝叓鐦ょ窗(x矛)鑳炰笉鍚岀▼搴﹀湴琛ㄩ仈(d谩)CD45鍜孋D45RO锛屽苟寮�(qi谩ng)鐑堣〃閬�(d谩)CD25銆傚ぇ

閮ㄥ垎ALK+ALCL鎮ｈ€呰〃閬�(d谩)涓婄毊绱�(x矛)鑳炶啘鎶楀師(epithelialmembraneantigen锛孍MA)鍜岀窗(x矛)鑳炴瘨椤嗙矑鐩搁棞(gu膩n)铔嬬櫧(TIA-1),椤嗙矑閰禕(granzymeB)锛岀┛瀛旂礌(perforin)銆侲BV绶ㄧ⒓鏍哥硸鏍搁吀(EBVencodedRNA锛孍BER)鍜屾經浼忚啘铔嬬櫧1(latentmembraneprotein1锛孡MP1)闄版€с€傚鏁�(sh霉)鏈塼(2;5)/NPM-ALK鏄撲綅鐨勬偅鑰�锛孉LK鐨勫厤鐤祫鍖栨煋鑹茬壒寰佹€у湴鍑虹従(xi脿n)鍦ㄨ優婕�銆佺窗(x矛)鑳炴牳鍜屾牳浠佷腑銆�

閫欐槸鐢变簬NPM涓嶅仠鍦扮┛姊簬鏍镐粊鑸囪優婕夸箣闁�锛屼綔鐐鸿級楂斿皣鏂板悎鎴愮殑铔嬬櫧璩�(zh矛)閬�(y霉n)杞�(zhu菐n)鑷虫牳浠併€侼PM甯舵湁瀵¤仛鍔熻兘鐨勭祼(ji茅)妲�(g貌u)鍩�锛屾甯告儏娉佷笅鏈冪櫦(f膩)鐢熻嚜韬殑瀵¤仛锛屼篃鍙互鑸嘚PM-ALK褰㈡垚鐣拌仛楂旓紝寰炶€屽皫(d菐o)鑷碞PM-ALK铔嬬櫧鍦ㄦ牳鍏�(n猫i)鐨勮仛闆�銆侫LK閭勫彲浠ヨ垏鍏跺畠浼欎即鍩哄洜鏄撲綅锛岀洰鍓嶅牨閬撶殑鍖呮嫭鏌撹壊楂�1锛�2锛�3锛�9锛�17锛�19锛�22锛屽潎鍙皫(d菐o)鑷碅LK鐨勭暟甯歌〃閬�(d谩)锛孉LK鍏嶇柅绲勫寲鏌撹壊鍙垎甯冨湪鑳炴伎銆佽優鏍告垨鍖呰啘绛変笉鍚屽崁(q奴)鍩熴€�

杓冪偤甯歌鐨勬湁t(1;2)(q25;p23)锛岃〃閬�(d谩)TPM3-ALK铔嬬櫧锛孉LK鏌撹壊灞€闄愬湪鑵槫绱�(x矛)鑳炵殑鑳炴伎锛屽苟涓旂窗(x矛)鑳炶啘鐨勬煋鑹叉洿寮�(qi谩ng)銆傝純鐐虹綍瑕嬬殑t(2;17)(p23;p23)锛岃〃閬�(d谩)CLTCL-ALK铔嬬櫧锛岀敱浜庤瀺鍚堣泲鐧戒腑鐨勭恫(w菐ng)鏍艰泲鐧介噸閺堣泲鐧藉弮鑸囦簡鍥婃场琛ㄩ潰缍�(w菐ng)鏍艰泲鐧借。鐨勬(g貌u)寤�锛岄’绀哄嚭鐛�(d煤)鐗圭殑椤嗙矑鐙€鑳炴伎鏌撹壊妯″紡銆�

浣嶄簬Xq11-12鐨凪SN鍩哄洜琚瓑瀵︾偤涓€鍊嬫柊鐨凙LK铻嶅悎鍩哄洜锛岀壒榛�(di菐n)鏄疉LK鏌撹壊灞€闄愪簬绱�(x矛)鑳炶啘銆傝€屽叾瀹冪殑鏄撲綅锛孉LK鏌撹壊鍦ㄨ優婕匡紝鍖呮嫭鐢眛(2;3)(p23;q11)锛宨nv(2)(p23q35)锛宼(2;19)(p23;p13)锛宼(2;17)(p23;q25)锛宼(2;22)(p23;q11_2)andt(2;9)(p23;q33)灏�(d菐o)鑷寸殑TFG-ALK锛孉TIC-ALK锛孴PM4-ALK锛孉LO17-ALK锛孧YH9-ALK锛孴RAF1-ALK銆�(瑕嬮檮琛�1)

2.琛€甯歌(gu墨)

ALCL鎮ｈ€呯櫧绱�(x矛)鑳炲楂�銆佽灏忔澘澧為珮銆丆RP澧為珮鍧囪純鐐哄父瑕�銆傞儴鍒嗘偅鑰咃紝鐗瑰垾鏄梾鐞嗙偤灏忕窗(x矛)鑳炰簽鍨嬬殑鎮ｈ€�锛屼镜瑗叉€у挤(qi谩ng)锛屽彲渚电姱楠ㄩ珦鍜屽鍛ㄨ锛屽嚭鐝�(xi脿n)鐧借鐥呯殑琛ㄧ従(xi脿n)銆�

4. 楠ㄩ珦甯歌(gu墨)

ALCL楠ㄩ珦渚电姱鐨勬偅鑰呬笉瓒�15%锛岄€氶亷楠ㄩ珦娲绘鍜岄€�(j矛n)琛屽厤鐤祫鍖栨煋鑹�锛屽彲鐛插緱姣旈楂撶┛鍒烘洿楂樼殑闄芥€х巼銆傚皯鏁�(sh霉)灏忕窗(x矛)鑳炰簽鍨嬬殑鎮ｈ€呬镜鐘楂撻仈(d谩)鍒扮櫧琛€鐥呴殠娈�锛屽彲閫�(j矛n)琛屾祦寮忕窗(x矛)鑳炶(sh霉)妾㈡煡銆傞倓鏈夊皯鏁�(sh霉)鎮ｈ€呭彲鍦ㄩ楂撲腑瑕嬪埌鍣鐝�(xi脿n)璞°€�

5. 娴佸紡绱�(x矛)鑳炴鏌�

鏈夌爺绌惰€呭皪19鍚岮LK+ALCL鐨勮叓鐦ょ祫绻旈€�(j矛n)琛屾祦寮忕窗(x矛)鑳炲垎鏋�锛岀壒榛�(di菐n)鐐猴細鑵槫绱�(x矛)鑳炲父杓冨ぇ锛孋D45bright锛屽湪CD45/SSC榛�(di菐n)鍦栦腑锛屾湁瑷卞绱�(x矛)鑳炵敋鑷冲鏁�(sh霉)绱�(x矛)鑳炶惤鍏ュ柈鏍哥窗(x矛)鑳炵殑鍗€(q奴)鍩�銆傛墍鏈夋偅鑰匔D30鍜孋D45闄芥€с€�

T绱�(x矛)鑳炴姉鍘熻〃閬�(d谩)鐨勯櫧鎬х巼鍒嗗垾鐐猴細CD2锛�67%;CD7锛�60%;CD3锛�45%;CD4锛�33%;CD5锛�14%;CD8锛�14%銆傚洜姝�锛屾祦寮忕窗(x矛)鑳炶(sh霉)鍙互鐢ㄤ簬鏈梾鐨勫厤鐤牳鍨嬫娓�锛屼絾闇€瑕佹敞鎰忚叓鐦ょ窗(x矛)鑳炴湁鍙兘钀藉埌娣嬪反绱�(x矛)鑳為杸浠ュ锛屼互鍏嶆紡瑷�銆�

姝ゅ锛屾湁鐨勭梾渚嬮倓琛ㄩ仈(d谩)CD13锛孋D56绛夛紝鍥犳闇€瑕佹敞鎰忚垏鍏跺畠椤炲瀷鐨勭櫧琛€鐥呯浉閼掑垾銆�

5.褰卞儚瀛�(xu茅)妾㈡煡

鎮ｈ€呴€�(j矛n)琛屾不鐧傚墠闇€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�锛屽畬鎴愬垎鏈�銆傞牳閮�銆佽兏閮ㄣ€佽吂閮�銆佺泦鑵擟T浣滅偤鍒嗘湡鐨勪緷鎿�(j霉)鎳�(y墨ng)甯歌(gu墨)妾㈡煡銆傚皪鏈夐鍙楃疮琛ㄧ従(xi脿n)鐨勬偅鑰�(濡傞鐥�锛岃叓鑴圭瓑)鍙仛楠ㄦ巸鎻忔鏌�銆�

濡傛偅鑰呮湁CNS鍙楃疮鐨勭櫏鐙€鎴栬〃鐝�(xi脿n)锛屾噳(y墨ng)閫�(j矛n)琛岄牠椤盡RI鎴朇T鎺冩弿銆傝繎骞翠締锛孭ET/CT宸查€愭几鎴愮偤鏈梾鍒嗘湡鍜屽啀瑭曚及鐨勯噸瑕佸伐鍏�銆�

6.寰皬鎾暎鍜屽井灏忔畼鐣欑梾妾㈡脯

鏈夊牨閬�锛孉LK+ALCL鎮ｈ€呮不鐧傚墠楠ㄩ珦鍙婂鍛ㄨ涓井灏忚叓鐦ゆ挱鏁�(MDD)鍜屾不鐧傚悗浠嶆湁寰皬鑵槫鎾暎(MRD)锛屽潎鑸囦笉鑹爯(y霉)鍚庢槑椤浉闂�(gu膩n)銆�

鐢变簬鍏掔90%鐨勬偅鑰呭潎鐐篘PM-ALK鍩哄洜铻嶅悎鎵€鑷�锛屽彧鏈夊皯閮ㄥ垎鎮ｈ€呮槸ALK鍩哄洜鑸囧叾瀹冧紮浼村熀鍥犵暟浣嶈瀺鍚堟墍鑷达紝鏁呴€氬父閲囧彇RT-PCR鐨勬柟娉曟娓偅鑰呴楂撳拰(鎴�)澶栧懆琛€涓璑PM-ALK铻嶅悎鍩哄洜鐨勮〃閬�(d谩)锛屼締妾㈡脯鍜岀洠(ji膩n)娓偅鑰呯殑MDD鍜孧RD銆�

浣嗗湪姹哄畾浣跨敤姝ゆ柟娉曞墠锛岄渶鍏堣瀵熸偅鑰呯梾鐞咥LK鐨勬煋鑹插垎甯冿紝濡傛灉ALK鐨勯櫧鎬ф煋鑹蹭笉鏄牳婕垮垎甯�锛屾彁绀篈LK鐨勪紮浼村熀鍥犱笉鏄疦PM锛岄偅涔堢洠(ji膩n)娓琋PM-ALK铻嶅悎鍩哄洜灏卞け鍘讳簡鎰忕京銆傞渶鍏堥噰鐢ㄤ簩浠ｆ脯搴忕瓑鏂规硶鏄庣⒑ALK鐨勪紮浼村熀鍥狅紝鍐嶉€氶亷浜屼唬娓簭鎴朢T-PCR鐨勬柟娉曚締鐩�(ji膩n)娓琈DD鍜孧RD銆�

(涓�)鍏掔ALK+ALCL鐨勮ê鏂锋(bi膩o)婧�(zh菙n)

1.鐥呯悊

绲勭箶鐥呯悊瀛�(xu茅)鍜屽厤鐤〃鍨嬩粛鏄噸瑕佺殑纰鸿ê渚濇摎(j霉)銆傞鍏堥渶瑕佺嵅寰楄冻澶犲ぇ鐨勬椿妾㈡ǎ鏈互瀹屾垚娣嬪反鐦ょ殑鍏ㄩ潰鍒嗘瀽锛屽寘鎷煋鑹查珨鍒嗘瀽锛孎(xi脿n)ISH鍜屽垎瀛愬熀鍥�?q奴)W鐮旂┒銆�

閫欎簺妾㈡煡灏嶄簬ALCL鑸囧弽鎳�(y墨ng)鎬ф垨鍏跺畠鑹€ф穻宸村鐢熸€х柧鐥�锛屼互鍙婇潪娣嬪反璧锋簮鐨勬儭鎬х柧鐥呯殑閼掑垾鍏锋湁閲嶈鐨勫児鍊�銆�

2.CNS渚电姱鐨勮ê鏂锋(bi膩o)婧�(zh菙n)

绗﹀悎浠ヤ笅浠讳綍涓€姊濆彲瑷烘柗涓绁炵稉(j墨ng)绯荤当(t菕ng)渚电姱:

(1)鑵﹁剨娑茬櫧绱�(x矛)鑳炶▓鏁�(sh霉)>5鍊�/μL锛屽苟浠ヨ叓鐦ょ窗(x矛)鑳炵偤涓�锛屽悓鏅傜磪绱�(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▓鏁�(sh霉)>5鍊�/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;

(2)椤辩缍�(j墨ng)楹荤椆锛屽嵆浣胯叇鑴婃恫涓劇鑵槫绱�(x矛)鑳�銆侀”鑵RI/CT鏈櫦(f膩)鐝�(xi脿n)鍗犱綅鎬х梾璁�;

(3)CT/MRI鍙鑵�銆佽剨楂撴垨鑵�銆佽剨鑶滅梾璁�;

(4)鑴婃煴楠ㄧ牬澹炴垨妞庢梺渚电姱寮曡捣鎺掑翱鐣板父銆佽偄楂旀椿鍕曢殰绀欑瓑绁炵稉(j墨ng)绯荤当(t菕ng)鐧ョ媭銆�

3.鑵槫绶╄В瑷烘柗妯�(bi膩o)婧�(zh菙n)

(1)瀹屽叏绶╄В(CR)锛氶€氶亷鏌ラ珨鍜屽奖鍍忓(xu茅)妾㈡煡璀夊鎵€鏈夌槫鐏跺叏閮ㄦ秷澶�銆侰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�(鏈€澶у緫绶�<1.5cm)锛屽悇鍣ㄥ畼褰卞儚瀛�(xu茅)鍙鍒扮殑鎵€鏈夌槫鐏跺潎宸叉秷澶�銆侾ET鎺冩弿宸茬劇闄芥€х櫦(f膩)鐝�(xi脿n)銆傞楂撴秱鐗�/娲绘姝ｅ父銆�

(2)閮ㄥ垎绶╄В(PR)锛氱槫鐏舵渶澶у緫绶氱府灏�≥50%锛屾矑鏈夋柊鐧�(f膩)鐦ょ伓銆�

(3)鐒″弽鎳�(y墨ng)(鐤剧梾绌�(w臎n)瀹�)(NR)锛氳⿻浼伴’绀烘矑鏈夐仈(d谩)鍒癙R锛屼絾涔熸矑鏈夋柊鐧�(f膩)鐦ょ伓銆�

(4)鐤剧梾閫�(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)鐦ょ伓鎴栭楂撳京(f霉)鐧�(f膩)銆�

(5)鐒℃硶纰哄畾鐨勫畬鍏ㄧ珐瑙ｏ細闆栫劧鐥呯伓鏈€澶у緫>1.5cm锛屼絾绺介珨涓婄梾鐏剁府灏�>75%(鐩镐簰鍨傜洿鍏╂鏈€澶ф┇寰�)銆傚緸褰卞儚瀛�(xu茅)涓婄劇娉曠⒑瀹氭槸鍚︾偤娈樼暀鐨勮叓鐦ゆ€х梾鐏�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�

(6)鍒嗗瓙鐢熺墿瀛�(xu茅)绶╄В(MCR)锛氶€氶亷RT-PCR鏂规硶鐩�(ji膩n)娓楂撴垨澶栧懆琛€铻嶅悎鍩哄洜ALK寰為櫧鎬ц綁(zhu菐n)鐐洪櫚鎬с€�

(7)鍒嗗瓙鐢熺墿瀛�(xu茅)寰�(f霉)鐧�(f膩)(鎴栭櫚杞�(zhu菐n)闄�)锛氭不鐧傚悗杞�(zhu菐n)鐐洪櫚鎬х殑ALK鍩哄洜鍐嶆妾㈡脯鐐洪櫧鎬�銆�

(鍥�)閼掑垾瑷烘柗

1.闁撹畩鎬уぇ绱�(x矛)鑳炴穻宸寸槫锛孉LK闄版€э細鏈梾鐐哄彟涓€绋瓹D30闄芥€х殑T绱�(x矛)鑳炴穻宸寸槫锛屽舰鎱�(t脿i)瀛�(xu茅)寰堥洠鑸嘇LK闄芥€х殑ALCL閼掑垾锛屽鏁�(sh霉)琛ㄩ仈(d谩)T绱�(x矛)鑳炵浉闂�(gu膩n)鐨勫厤鐤(bi膩o)瑷�锛屼絾ALK闄版€�銆傛湰鐥呭瑕嬩簬鎴愪汉(40锝�65姝�)銆�

2.ALK闄芥€х殑褰屾极澶绱�(x矛)鑳炴穻宸寸槫锛氭湰鐥呴潪甯哥綍瑕�,涓昏渚电姱娣嬪反绲�(ji茅)鎴栬〃鐝�(xi脿n)鐐虹副闅旇叓鐗�锛岄蓟鍜�銆侀銆佽粺绲勭箶銆佽儍绛夌祼(ji茅)澶栦镜鐘篃鏈夊牨閬�銆�

鏈梾涔熸湁ALK铻嶅悎铔嬬櫧琛ㄩ仈(d谩)锛屽鐐哄眬闄愪簬鑳炴伎鐨勯绮掔媭鏌撹壊妯″紡锛屾彁绀虹偤CLTC-ALK铔嬬櫧;灏戞暩(sh霉)鐐篘PM-ALK铔嬬櫧銆傚挤(qi谩ng)琛ㄩ仈(d谩)EMA鍜屾伎绱�(x矛)鑳炴(bi膩o)瑷楥D138鍜孷S38銆侰D45寮遍櫧鎴栭櫚鎬с€傜櫧绱�(x矛)鑳炵郴鎶楀師CD3锛孋D20锛孋D79a锛孭ax-5闄版€�銆侰D30闄版€�銆傛湰鐥呬镜瑗叉€у挤(qi谩ng)锛屽寲鐧傛晥鏋滀笉浣�锛岄爯(y霉)鍚庡樊銆�

3.鍘熺櫦(f膩)鐨啔鐨勯枔璁婃€уぇ绱�(x矛)鑳炴穻宸寸槫锛氬湪鍏掔闈炲父缃曡锛屽鐐哄€嬩緥鍫遍亾銆傜梾鐞嗗舰鎱�(t脿i)瀛�(xu茅)鑸嘇LK+ALCL鐩镐技锛屼絾浠ラ檺灞€鎬х毊鑶氭悕瀹崇偤涓�銆�

鑵槫绱�(x矛)鑳炴湁婵€娲荤殑CD4+T绱�(x矛)鑳炴牳鍨嬶紝涓嶅悓绋嬪害鍦颁笩澶盋D2锛孋D5鍜�(鎴�)CD3锛岀稉(j墨ng)甯歌〃閬�(d谩)绱�(x矛)鑳炴瘨铔嬬櫧銆�5%鑰呯偤CD8+T绱�(x矛)鑳炴牳鍨�銆�75%浠ヤ笂鐨勮叓鐦ょ窗(x矛)鑳炶〃閬�(d谩)CD30銆傝垏绯荤当(t菕ng)ALCL涓嶅悓锛屽鏁�(sh霉)C-ALCL琛ㄩ仈(d谩)鐨啔娣嬪反绱�(x矛)鑳炴姉鍘�(CLA)锛屼絾涓嶈〃閬�(d谩)EMA;ALK闄版€у瑕�銆傛湰鐥呴€�(j矛n)灞曠珐鎱�锛岄爯(y霉)鍚庤純濂�銆�

4.绲�(ji茅)绡€(ji茅)纭寲鍨嬮湇濂囬噾娣嬪反鐦わ細閮ㄥ垎ALCL鍙瓨鍦ㄧ‖鍖栫従(xi脿n)璞�锛屼篃鐐篊D30闄芥€�锛屾槗瑾よê鐐洪湇濂囬噾娣嬪反鐦ゃ€備絾鐢变簬ALCL鏄竴鍊婽绱�(x矛)鑳炰締婧愮殑鑵槫锛岃€岄湇濂囬噾娣嬪反鐦ゆ槸B绱�(x矛)鑳炰締婧愮殑鑵槫锛岄€氶亷鍋欳D15銆乸an-B銆乸an-T鎶楀師銆丒MA銆丳AX-5/BSAP銆丄LK铔嬬櫧锛屾姉鍘熷彈楂斿熀鍥犻噸鎺�锛屼簩鑰呰純鏄撳崁(q奴)鍒�銆�

5.鎰熸煋鎬х柧鐥咃細鐢变簬鏈梾澶氭湁鐧�(f膩)鐔�锛岀槫鐏堕儴浣嶅彲浼存湁绱呰叓鐔辩棝锛岃甯歌(gu墨)鍙〃鐝�(xi脿n)鐐虹櫧绱�(x矛)鑳炲崌楂橈紝CRP鍗囬珮锛屼笖鏃╂湡鍗冲彲鍑虹従(xi脿n)鑲�銆侀绛夌祼(ji茅)澶栦镜鐘紝鏁呮槗鑸囨劅鏌撴€х柧鐥呮贩娣�銆�

纰鸿ê鍓嶅線寰€鍏堣瑷虹偤娣嬪反绲�(ji茅)鐐�銆佽渹绐╃箶鐐庛€佽偤鐐�銆佺祼(ji茅)鏍搞€侀楂撶値绛�銆傛晠闇€鍔犲挤(qi谩ng)灏嶆湰鐥呯殑瑾�(r猫n)璀橈紝灏嶄簬鎶楁劅鏌撴晥鏋滀笉浣�锛屾垨鏈夊鍣ㄥ畼鍙楃疮鐨勬偅鑰呯⿳妤佃绲勭箶娲绘鏄庣⒑瑷烘柗銆�

鍥�銆佽嚚搴婂嵄闅害鍒嗗堡

鐢变簬ALCL鐧�(f膩)鐥呯巼浣庯紝闆栫稉(j墨ng)鍦嬪収(n猫i)澶栧瀹跺崝(xi茅)浣滅祫鐮旂┒锛岀洰鍓嶅皻鐒℃槑纰虹当(t菕ng)涓€鐨勯爯(y霉)鍚庝笉鑹浉闂�(gu膩n)鍥犵礌銆傚皪浜庡厭绔ユ偅鑰�锛屽牨閬撶殑鑸囨不鐧傚け鏁楃浉闂�(gu膩n)鐨勫洜绱犱富瑕佸寘鎷細

鈶犺嚚搴婂洜绱�锛屽鍑虹従(xi脿n)绺遍殧渚电姱锛屽収(n猫i)鑷�(鑲猴紝鑲�锛岃劸)渚电姱锛岀毊鑶氫镜鐘�;

鈶＄梾鐞嗕簽鍨嬩腑鐨勬穻宸寸祫绻旂窗(x矛)鑳炰簽鍨嬪拰灏忕窗(x矛)鑳炰簽鍨�;

鈶㈣ê鏂锋檪鐢≒CR鏂规硶妾㈡脯澶栧懆琛€鍜�(鎴�)楠ㄩ珦NPM-ALK浣滅偤寰皬鎾暎鐤剧梾(minimaldisseminateddisease锛孧DD)鎸囨(bi膩o)鐐洪櫧鎬�;

鈶ｄ竴鐧傜▼鍖栫檪鍚庣敤PCR妾㈡脯琛€/楠ㄩ珦NPM-ALK浣滅偤寰皬娈樼暀鐥�(minimalresidualdisease锛孧RD)鎸囨(bi膩o)浠嶇偤闄芥€�銆�

浜�銆佹不鐧�

(涓€)绯荤当(t菕ng)鍖栫檪

1.鍖栫檪鍘熷墖

鐩墠鍦嬮殯涓婂厭绔LCL鐨勬不鐧傚師鍓囧苟涓嶇当(t菕ng)涓€锛屾湰寤鸿鎻愪緵鐨勬不鐧傛柟妗堝弮鑰僂ICNHL-ALCL99銆傜敱浜庢湁鍫遍亾锛屽湪楠ㄩ珦绉绘鍚庡京(f霉)鐧�(f膩)鐨勬偅鑰�锛屽儏闀锋槬鑺卞牽(VBL)6mg/(m²·w)娌荤檪浠嶅緱鍒伴暦鏈熺殑鎸佺簩(x霉)绶╄В锛屽叾浣滅敤涓嶅儏鏄洿鎺ユ鍌疯叓鐦ょ窗(x矛)鑳�锛岄倓鍙互瑾樺皫(d菐o)妯圭獊鐙€绱�(x矛)鑳炵殑鎴愮啛锛屾仮寰�(f霉)姗�(j墨)楂旀姉鑵槫鐨勫厤鐤弽鎳�(y墨ng)鍜屾姉琛€绠＄瓑鍔熻兘銆傚洜姝�锛屾垜鍊戞帹钖︾殑鏂规澧炲姞浜嗛暦鏄ヨ姳鍫跨殑缍寔娌荤檪銆傚悇閱�(y墨)闄㈡牴鎿�(j霉)鍚勮嚜鎯呮硜閬告搰鎳�(y墨ng)鐢�銆傚厑瑷卞皪鎵€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�銆傚洜闀锋槬鏂板牽銆侀暦鏄ュ湴杈涚劇娉曟浛浠ｉ暦鏄ヨ姳鍫跨殑浣滅敤锛屽苟涓嶅缓璀版浛鎻涖€�

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�锛屽寘鎷細楠ㄩ珦銆佽兏鑵规按銆佹泛琛ㄦ穻宸寸槫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�銆佺槫鐏舵墜琛�(sh霉)鍙栨椿妾€€傜劧鍚庡畬鍠勮叓鐦ょ窗(x矛)鑳炵殑MICM鍒嗗瀷銆傚畬鎴愪腑蹇冪梾鐞嗘渻瑷�(鑷冲皯2瀹朵笁鐢查啱(y墨)闄㈢梾鐞嗚€佸斧鏈冭ê)銆�

(2)寤鸿鑳搁鍙婇珎楠ㄥ叐鍊嬮儴浣嶉楂撶┛鍒烘垨娲绘锛氭噳(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〃鍨嬪垎鏋愩€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€佽倽鑵庡姛鑳�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井閲忕窗(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˙瓒呫€佸彲鐤戝彈渚甸儴浣嶇殑楠ㄩ鐗�銆丆T绛夊奖鍍忓(xu茅)妾㈡煡浠ヤ究浜嗚В鐥呰畩鑼冨湇銆傛嚪鐤戜腑妯炵缍�(j墨ng)绯荤当(t菕ng)鐥呰畩鏅傚彲琛岃叇鍙婅剨楂揗RI妾㈡煡銆傛湁姊濅欢鐨勮PET/CT妾㈡煡銆�

(6)楠ㄩ珦鍙婂鍛ㄨ鏌PM-ALK铻嶅悎鍩哄洜(濡傛灉鐥呯悊椤ずALK鏌撹壊涓嶆槸鏍告伎鍒嗗竷锛屼笉鍋氭妾㈡脯)銆�

(7)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�銆佸偝鏌撶梾绛夌媭娉併€�

3.鎸夊嵄闅害鍒嗙祫

A绲勶細瀹屽叏鍒囬櫎鐨処鏈�銆�

B绲勶細闋�(y霉)鍚庡ソ鐨勪竴绲�銆�

-娌掓湁鐨啔渚垫饯

-娌掓湁绺遍殧鍙楃疮

-鐥呯悊娌掓湁娣嬪反绲勭箶绱�(x矛)鑳炶畩鐣扮殑璀夋摎(j霉)锛岄潪灏忕窗(x矛)鑳炶畩鐣颁簽鍨�

-楠ㄩ珦娌掓湁鍣鐝�(xi脿n)璞�锛屼笉鍚堝苟鍣绱�(x矛)鑳炵稖鍚堝緛

-闈濧LCL鐧借鐥呴殠娈�

-楠ㄩ珦鍜屽鍛ㄨNPM-ALK(-)

C绲勶細闋�(y霉)鍚庡樊鐨勪竴绲勶紝鍖呮嫭浠ヤ笅鐗归粸(di菐n)鐨勭梾浜�

-鐨啔娲绘璀夊鏈夌毊鑶氭悕瀹�(涓嶆槸I鏈�)

-鏈夌副闅斿拰(鎴�)鑲鸿嚐鍙楃疮

-鐥呯悊鏈夋穻宸寸祫绻旂窗(x矛)鑳炶畩鐣�锛屾垨鐐哄皬绱�(x矛)鑳炶畩鐣颁簽鍨�

-楠ㄩ珦鍙鍣鐝�(xi脿n)璞�锛屾垨鍚堝苟鍣绱�(x矛)鑳炵稖鍚堝緛

-ALCL鐧借鐥呴殠娈�

-楠ㄩ珦鎴栧鍛ㄨNPM-ALK(+)

D绲勶細鏈塁NS鍙楃疮鐨勭梾浜�銆�

4.鍖栫檪鏂规

(1)鏂规娴佺▼

A绲勶細鎸夌収CourseP銆丆ourseAV1銆丆ourseBV1銆丆ourseAV2鏂规闋嗗簭閫�(j矛n)琛屻€�

B绲勶細鎸夌収CourseP銆丄V1銆丅V1銆丄V2銆丅V2銆丄V3銆丅V3鏂规闋嗗簭閫�(j矛n)琛屻€備簬CourseP娌荤檪绗�5澶╄⿻浼版不鐧傚弽鎳�(y墨ng)銆侫V2鏂规鍖栫檪鍚庤⿻浼扮珐瑙ｆ儏娉�銆侭V3鏂规鍖栫檪鍚庡啀瑭曚及锛岃嫢鎸佺簩(x霉)瀹屽叏绶╄В锛岀董鎸佹柟妗堟噳(y墨ng)鐢ㄩ暦鏄ヨ姳鍫�锛屾瘡鍛�1娆�锛屽叡12鏈�;鑻ユ湁娈樼暀鐥呯伓锛屾洿鎻涙柟妗�銆�

C绲勶細鎸夌収CourseP銆丄V1銆丅V1銆丄V2銆丅V2銆丄V3銆丅V3鏂规闋嗗簭閫�(j矛n)琛�銆備簬CourseP娌荤檪绗�5澶╄⿻浼版不鐧傚弽鎳�(y墨ng)銆侫V2鏂规鍖栫檪鍚庤⿻浼扮珐瑙ｆ儏娉�銆侭V3鏂规鍖栫檪鍚庡啀瑭曚及锛岃嫢鎸佺簩(x霉)瀹屽叏绶╄В锛岀董鎸佹柟妗堟噳(y墨ng)鐢ㄩ暦鏄ヨ姳鍫�锛屾瘡鍛�1娆★紝鍏�24鏈�;鑻ユ湁娈樼暀鐥呯伓锛屾洿鎻涙柟妗�銆�

D绲勶細鏈塁NS鍙楃疮鐨勭梾浜哄彲鍙冮柋鎴愮啛B绱�(x矛)鑳炴穻宸寸槫C绲勬湁CNS鍙楃疮鐨勬柟妗堝寲鐧�?锛涱嚖鐔僘(ji茅)鏉熷悗鐢ㄩ暦鏄ヨ姳鍫挎瘡鍛�1娆＄董鎸�24鏈堛€�

(2)鍏烽珨鏂规

鈶燙ourseP鏂规

鍦板绫虫澗(Dex)5mg/(m²·d)锛�1澶�1娆�锛岄潨鑴堟淮娉ㄦ垨鑰呭彛鏈�锛宒1锝瀌2;Dex10mg/(m²·d)锛�1澶�2娆★紝闈滆剤婊存敞鎴栬€呭彛鏈�锛宒3锝瀌5;鐠�(hu谩n)纾烽叞鑳�(CTX)200mg/(m²·d)锛�15鍒嗛悩闈滆剤婊存敞锛宒1锝瀌2銆傞灅娉�(涓夎伅(li谩n))d1(鍏烽珨瑕嬮檮琛�2)銆傛敞锛氬寲鐧傚悓鏅傛按鍖�銆佸牽鍖�锛屽苟鍙彛鏈嶅垾鍢岄唶闄嶄綆灏块吀姘村钩锛岀浜斿ぉ鍋氱槫鐏惰⿻浼�銆�

鈶ourseAV1/AV2/AV3

绗竴鐧傜▼闁嬪浜庡寲鐧傜殑绗�6澶╋紝鑻NC>0.5×10⁹/L锛孭LT>50×10⁹/L锛岄毃鍚庣殑鐧傜▼闁嬪浜庡墠1鍊嬬檪绋嬬殑绗�21澶┿€侱ex10mg/(m²·d)锛�1澶�2娆�锛岄潨鑴堟淮娉ㄦ垨鍙ｆ湇d1锝瀌5;鐢叉皑鍠嬪懁(MTX)3g/m²闈滆剤婊存敞3灏忔檪锛宒1;浜炶憠閰搁垼(CF)15mg/m²闈滆剤婊存敞锛岀敤MTX鍚�24灏忔檪闁嬪锛�48灏忔檪娓琈TX琛€婵冨害姣�6灏忔檪瑙ｆ晳涓€娆�锛岀洿鑷矼TX婵冨害<0.15μmol/L锛屽墖涓嶅啀瑙ｆ晳銆�

闉樻敞(涓夎伅(li谩n))d2;鐢∕TX鍚�24灏忔檪銆傜暟鐠�(hu谩n)纾烽叞鑳�(IFO)800mg/m²锛岄潨鑴堟淮娉�1灏忔檪锛宒1锝瀌5;绗竴澶╃郸钘ヤ簬MTX鍓嶉潨鑴堟淮娉�锛屽悓鏅備簣缇庡徃閳�(Mesna)330mg/(m²·娆�)锛岀敤鐣扮挵(hu谩n)纾烽叞鑳虹殑0h銆�4h銆�8h闈滆剤婊存敞;闃跨硸鑳炶嫹(Ara-C)150mg/(m²·娆�)锛岄潨鑴堟淮娉�1灏忔檪锛孮12h锛宒4锝瀌5;

渚濇墭娉婅嫹(VP16100mg/m²锛岄潨鑴堟淮娉�2灏忔檪锛宒4锝瀌5(鍦ˋra-c鍚庣郸浜�)锛孷P16鏈€楂樼█閲嬪€嶆暩(sh霉)鐐�0.4mg/mLNS:VP16=2.5mL:1mg;闀锋槬鑺卞牽(VBL)6mg/m²(鏈€澶ч噺涓嶈秴閬�10mg)锛岄潨鑴堟淮娉�锛宒1銆傛敞锛歁TX銆丆TX鎳�(y墨ng)鐢ㄦ檪瑕佹按鍖栥€佸牽鍖�銆侫V1鏂规婵€绱犻渶5澶╂笡鍋�銆�

鈶ourseBV1/BV2/BV3

鑻NC>0.5×10⁹/L锛孭LT>50×10⁹/L锛岄毃鍚庣檪绋嬮枊濮嬩簬鍓�1鐧傜▼鐨勭21澶┿€侱ex10mg/(m²·d)锛屼竴澶╁叐娆�锛屽彛鏈嶆垨闈滆剤婊存敞锛宒1锝瀌5;MTX3g/m²闈滆剤婊存敞3灏忔檪锛宒1CF15mg/m²闈滆剤婊存敞锛岀敤MTX鍚�24灏忔檪闁嬪锛屾瘡6灏忔檪瑙ｆ晳涓€娆�锛�48灏忔檪娓琈TX琛€婵冨害锛岀洿鑷矼TX婵冨害<0.15μmol/L锛屽墖涓嶅啀瑙ｆ晳銆�

闉樻敞(涓夎伅(li谩n))d2锛岀敤MTX鍚�24灏忔檪;鐠�(hu谩n)纾烽叞鑳�(CTX)200mg/m²锛岄潨鑴堟淮娉�60鍒嗛悩锛宒1锝瀌5锛岀涓€澶╃郸钘ヤ簬MTX鍓�;鏌旂磪闇夌礌(DNR)25mg/m²锛岄潨鑴堟淮娉�6灏忔檪鍏ワ紝d4锝瀌5;VBL6mg/m²(鏈€澶ч噺涓嶈秴閬�10mg)闈滄帹锛宒1銆傛敞锛歁TX锛孋TX鎳�(y墨ng)鐢ㄦ檪瑕佹按鍖�銆佸牽鍖�銆�

鈶BL缍寔娌荤檪

VBL6mg/m²(鏈€澶ч噺涓嶈秴閬�10mg)闈滄帹锛屾瘡鍛�1娆�锛孊绲勭董鎸�12鏈�;C绲勭董鎸�24鏈堛€備腑鏈熻⿻浼版垨缍寔鍓嶈⿻浼颁粛鏈夋畼鐣欑梾鐏跺拰(鎴�)涓湡瑭曚及鍙婁互鍚嶯PM-ALK鐐�(+)锛孷BL缍寔鑷冲皯鑷�2骞�銆傛敞锛氱1娆＄董鎸佺敤钘ヨ垏CourseBV3闁撻殧21澶�銆�

鎳�(y墨ng)鐢╒BL鏅�锛屾敞鎰忕缍�(j墨ng)绯荤当(t菕ng)鐧ョ媭鍙婅甯歌(gu墨)锛屾噳(y墨ng)淇濊瓑ANC>0.5×10⁹/L锛孭LT>50×10⁹/L銆傜敤钘ラ亷绋嬩腑濡傚嚭鐝�(xi脿n)楠ㄩ珦鎶戝埗(涓€х矑绱�(x矛)鑳炲皬浜�0.5×10⁹/L)锛屽彲閬╃暥(d膩ng)娓涗綆VBL鍔戦噺1/31/2锛屽悎骞舵劅鏌撳彲鏆檪鍋滅敤VBL銆傛劅鏌撴帶鍒�銆佽璞℃仮寰�(f霉)鍚庣洝鏃╂仮寰�(f霉)鐢ㄨ棩銆�

鈶ゆ偅鑰呯董鎸佹湡闁揅NS杞�(zhu菐n)绉婚爯(y霉)闃�

闆栫劧鏈梾CNS鐧�(f膩)鐥呯巼杓冧綆锛屼絾浠嶆湁鍦╒BL缍寔鏈熼枔鐧�(f膩)鐢熻叇杞�(zhu菐n)绉荤殑鍫遍亾锛屽洜姝ゅ皪浜庝笅杩伴鍨嬫偅鑰呭湪VBL缍寔鏈熼枔寤鸿姣�3鍊嬫湀绲︿簣涓夎伅(li谩n)闉樻敞1娆★細ALCL鍦ㄥ垵娆℃不鐧備腑閫�(j矛n)灞�;鍒濇娌荤檪鍚�3鍊嬫湀鍏�(n猫i)鏃╂湡寰�(f霉)鐧�(f膩);闆栭潪鏃╂湡寰�(f霉)鐧�(f膩)锛屼絾鏈塁NS寰�(f霉)鐧�(f膩)锛屾垨鍒濇不浼存湁CNS渚电姱鐨勬偅鑰�;PCR鏂规硶妾㈡脯澶栧懆琛€鎴栭楂揘PM-ALK鎸佺簩(x霉)闄芥€�銆�

5.鐧傛晥瑭曚及

(1)鑷ㄥ簥瑙€瀵熸寚妯�(bi膩o)

鈶犲寲椹楁寚妯�(bi膩o)锛氳甯歌(gu墨)+CRP銆佺敓鍖栧叏闋�銆佸嚌琛€鍔熻兘銆侀惖铔嬬櫧銆乻CD25銆佺窗(x矛)鑳炲洜瀛�銆侀楂撳櫖琛€鐝�(xi脿n)璞�(濡傞楂撲腑鏈夎叓鐦ょ窗(x矛)鑳炴垨鍣鐝�(xi脿n)璞￠渶寰�(f霉)鏌ヨ嚦娑堝け)銆佸鍛ㄨ鍜岄楂揘PM-ALK銆佽叇鑴婃恫銆�

鈶″奖鍍忓(xu茅)鎸囨(bi膩o)锛氱槫鐏跺奖鍍忓(xu茅)瑭曚及锛岄渶琛岀槫鐏剁稉(j墨ng)绶氭脯閲�锛岃垏鍓嶉潰灏嶆瘮銆�

鈶ET/CT锛氬鏋滃闀锋浠跺厑瑷�锛屽彲浠ュ湪鍖栫檪鍓嶃€佷腑鏈熻⿻浼板強鏀剧檪鍓嶉€�(j矛n)琛�銆�

(2)鐧傛晥瑭曚及

鈶燚5瑭曚及锛氫富瑕佸仛妾㈡脯涔嬬槫鐏剁殑B瓒呭拰(鎴�)CT锛屾脯閲忕槫鐏跺ぇ灏�锛岃▓绠楃府灏忕殑%銆�

瑭曚及妯�(bi膩o)婧�(zh菙n)锛氱槫鐏剁府灏忊墽75%锛岀偤妤靛叾鏁忔劅锛�

鐦ょ伓绺皬>25%锛�<75%鐐烘晱鎰燂紝

鐦ょ伓绺皬鈮�25%锛岀偤涓嶆晱鎰�

鐦ょ伓绺皬≤25%锛孉绲勩€丅绲勬偅鑰呴渶鍗囩礆鑷矯绲�銆�

鈶′腑鏈熻⿻浼帮細绗�4鐧傜▼鍚庤⿻浼�锛屽寘鎷�

鍏ㄨ韩鐦ょ伓鐨勭鏌ワ細鏍规摎(j霉)鐥呭垵鐨勭槫鐏舵儏娉佹焙瀹氭鏌ュ収(n猫i)瀹�锛屼富瑕佺湅鏈夌劇娈樼暀鐥呯伓锛屼腑妯炰镜鐘繀闋堣闋”鎴栬剨楂揗RI锛岀洝鍙兘鍋歅ET/CT銆傜浉闂�(gu膩n)琛€娓呭(xu茅)妾㈡煡锛氳倽鑵庡姛鑳�銆佸績鑲岄叾妾㈡脯銆佽叓鐦NA銆丩DH銆佸翱閰搞€丆RP銆侀惖铔嬬櫧;楠ㄩ珦鍙婂鍛ㄨNPM-ALK銆傞楂撳拰鑵﹁剨娑茬殑褰㈡厠(t脿i)鍙婃畼鐣欑梾妾㈡煡(涓嶇鏄惁鏈夐楂撳拰CNS渚电姱鐨勭梾浜�)銆�

瑭曚及妯�(bi膩o)婧�(zh菙n)锛氬鑳介仈(d谩)鍒伴儴鍒嗙珐瑙�锛屽彲浠ョ辜鍘熸柟妗堟不鐧�锛屽悓鏅傚瘑鍒囪瀵�銆傚娌荤檪鐒℃晥鎴栭€�(j矛n)灞�锛岄渶鎻涚敤鍏跺畠娌荤檪鏂规銆�

鈶㈠悗鏈熻⿻浼帮細BV3鍚�锛岃垏涓湡妾㈡煡椤炰技锛屼腑鏈熻⿻浼版湁鍟忛鐨勯爡鐩噸榛�(di菐n)鏌ャ€傛湁浠讳綍娈樼暀鐥呯伓鑰�锛屽彲鍋氫簩娆℃椿妾㈡垨PET/CT鏄庣⒑銆傚鑳介仈(d谩)鍒伴儴鍒嗙珐瑙ｏ紝鍙互閫�(j矛n)鍏ョ董鎸佹不鐧�锛屽悓鏅傚瘑鍒囪瀵�銆傚娌荤檪鐒℃晥鎴栭€�(j矛n)灞�锛岄渶鎻涚敤鍏跺畠娌荤檪鏂规銆�

鈶ｅ仠钘ュ墠妾㈡煡锛氬寘鎷嚐鍣ㄥ姛鑳姐€佺槫鐏跺強鍌虫煋鐥呮簮鐨勬鏌�銆�

鐢熷寲妾㈡脯锛氳倽鑵庡姛鑳�銆丩DH

鐥呮瘨绡╂煡锛氱敳涔欎笝涓佹垔鑲濇姉楂�銆丆MV銆佹姣�銆佽壘婊嬬梾绛�

蹇冭嚐锛氬績闆诲湒銆佸績鑷熷僵瓒�

鑵归儴锛氳倽鑵庡姛銆佹穩绮夐叾銆佽吂閮˙瓒�

CNS锛氶牠椤盡RI锛岃剨楂撳崰浣嶆噳(y墨ng)鍋氱浉鎳�(y墨ng)閮ㄤ綅MRI

鐪肩潧锛氳鍔�銆佺溂搴�

鍏嶇柅鍔熻兘锛欳D绯诲垪銆両g绯诲垪

楠ㄩ珦甯歌(gu墨)锛�

鑵﹁剨娑叉鏌ワ細甯歌(gu墨)銆佹秱鐗�銆佹祦寮忕窗(x矛)鑳炴鏌�

鐦ょ伓瑭曚及锛氱浉闂�(gu膩n)閮ㄤ綅B瓒呫€丆T妾㈡煡銆丮RI妾㈡煡

楠ㄩ珦鍙婂鍛ㄨNPM-ALK(濡傜梾鐞咥LK鏌撹壊涓嶆槸鏍告伎鍒嗗竷锛屼笉鐢ㄥ仛姝ゆ娓�)

濡傝⿻浼扮祼(ji茅)鏋滈仈(d谩)鍒拌叓鐦ゅ畬鍏ㄧ珐瑙ｅ拰鍒嗗瓙鐢熺墿瀛�(xu茅)瀹屽叏绶╄В锛屽彲鍋滆棩銆�

(3)闅ㄨí锛氶毃瑷嚦5骞�

鈶犲仠钘ョ涓€銆佷簩骞达細姣�3鍊嬫湀涓€娆＄啊鍠⿻浼�锛屾瘡娆″仛鐦ょ伓鐨勫奖鍍�(B瓒呯偤涓绘垨CT骞虫巸)銆佽倽鍔�銆丩DH锛岄楂撴垨澶栧懆琛€NPM-ALK銆傛瘡6鍊嬫湀涓€娆″ぇ瑭曚及锛屾瘡娆″仛鐦ょ伓鐨勫奖鍍�(B瓒�銆佸寮�(qi谩ng)CT妾㈡煡鎴朚R)銆佸厤鐤姛鑳�銆佽倽鍔熷拰LDH銆侀绌�(濡傛灉鏈夐楂撲镜鐘�)锛岄楂撴垨澶栧懆琛€NPM-ALK绛�銆傚仠钘�3鍊嬫湀棣栨妾㈡煡锛氱槫鐏舵鏌ユ渶濂藉仛PET/CT銆�

鈶＄涓夊勾浠ュ悗锛氭瘡鍗婂勾瑭曚及涓€娆�锛屼富瑕佸仛鐦ょ伓鐨勫奖鍍�(B瓒呭拰CT骞虫巸)鍙婅倽鍔熷拰LDH銆傝鎯呮硜鍔犲仛鍏�(n猫i)鍒嗘硨婵€绱犲強鏅哄晢绛夋鏌�銆�

(浜�)閫犺骞茬窗(x矛)鑳炵Щ妞�

ALCL寰�(f霉)鐧�(f膩)鐨勬偅鑰呭鏁�(sh霉)灏嶆惗鏁戞€ф不鐧傚弽鎳�(y墨ng)鑹ソ锛岃嚜楂旈€犺骞茬窗(x矛)鑳炵Щ妞�(auto-HSCT)鍙婄暟鍩哄洜閫犺骞茬窗(x矛)鑳炵Щ妞�(allo-HSCT)鍧囨湁鎴愬姛娌荤檪闆ｆ不寰�(f霉)鐧�(f膩)ALCL鐨勫牨閬�銆備絾娌荤檪涓€�(j矛n)灞�銆佹垨鍋滆棩鍚庝笁鍊嬫湀鍏�(n猫i)寰�(f霉)鐧�(f膩)銆佹垨瑷烘柗鏅傜梾鐞嗗厤鐤祫鍖朇D3鏌撹壊闄芥€х殑鎮ｈ€�锛宎llo-HSCT鐨勬不鐧傛晥鏋滃劒(y艒u)浜巃uto-HSCT銆�

(涓�)鍒嗗瓙闈跺悜钘ョ墿娌荤檪

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