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(涓€)鑷ㄥ簥琛ㄧ従(xi脿n)

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3.鐨柟锛氬妯�锛屽彲鐐哄叏韬枒涓樼柟銆佺磪鏂�銆佹按鑵€侀夯鐤规ǎ鐨柟銆佽剛鑶滅値绛夈€�

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5.璨ц銆佸嚭琛€锛氬嚭琛€鍖呮嫭鐨啔榛忚啘銆佺┛鍒洪儴浣嶄互鍙婃秷鍖栭亾銆佽偤銆佷腑妯炵瓑鍏�(n猫i)鑷熷嚭琛€銆傝钵琛€鍓囩敱鍑鸿浠ュ強绱�(x矛)鑳炲洜瀛愭姂鍒堕楂撻€犺鎵€鑷淬€�

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2.楠ㄩ珦璞★細鏃╂湡鍣绱�(x矛)鑳炲苟涓嶅父瑕�锛岃垏鑷ㄥ簥琛ㄧ従(xi脿n)鐨勫毚(y谩n)閲� 绋嬪害涓嶇浉骞宠锛屽儏琛ㄧ従(xi脿n)鐐哄弽鎳�(y墨ng)鎬х祫绻旂窗(x矛)鑳炲鐢燂紝鐒℃儭鎬х窗(x矛)鑳� 娴告饯锛屾櫄鏈熷櫖琛€鐝�(xi脿n)璞￠櫧鎬х巼楂樸€傞楂撳収(n猫i)鏈櫦(f膩)鐝�(xi脿n)鍣绱�(x矛)鑳炰笉鑳� 鎺掗櫎HLH锛屾噳(y墨ng)瀵嗗垏绲�(ji茅)鍚堣嚚搴�銆�

3.鑲濆姛鑳斤細鍙〃鐝�(xi脿n)鏈変綆鐧借泲鐧借鐧�锛岃娓呰綁(zhu菐n)姘ㄩ叾涓嶅悓绋� 搴﹀崌楂樻垨鑶界磪绱犲崌楂�锛岃垏鑲濊嚐鍙楃疮绋嬪害涓€鑷�銆�

4.鍑濊鍔熻兘锛氬湪鐤剧梾娲诲嫊鏈燂紝甯告湁鍑濊鍔熻兘鐣板父锛屼綆绾栫董铔嬬櫧鍘熻鐧�锛屾椿鍖栭儴鍒嗗嚌琛€娲婚叾鏅傞枔(APTT)寤堕暦锛屽嚌琛€閰跺師鏅傞枔(PT)寤堕暦銆�

5.鑴傞浠ｈ瑵锛氱梾绋嬫棭鏈熷嵆鍙嚭鐝�(xi脿n)楂樼敇娌逛笁閰鐧�锛屾澶栧彲鏈変綆瀵嗗害鑴傝泲鐧藉楂樺拰楂樺瘑搴﹁剛铔嬬櫧娓涗綆銆�

6.绱�(x矛)鑳炲洜瀛愭績搴︼細鍕曟厠(t脿i)鐩�(ji膩n)娓窗(x矛)鑳炲洜瀛愭按骞冲彲浠ュ垽鏂风柧鐥呭毚(y谩n)閲嶇▼搴﹀強娲诲嫊鎯呮硜?锛熸墖鑻勮āD25(sCD25)鍗冲彲婧舵€L-2鍙楅珨α閺堟槑椤崌楂樻槸瑷烘柗HLH鐨勯噸瑕佹(bi膩o)婧�(zh菙n)涔嬩竴锛岃€冩叜鍒板悇瀵﹂瀹ら枔鐨勮宸篃鍙皣sCD25>鍧囨暩(sh霉)±2SD瑕栫偤鏈夎ê鏂锋剰缇┿€傚叾浠栫窗(x矛)鑳炲洜瀛愬IFN-γ銆両L-10鎴朓L-6绛変篃鍙槑椤崌楂�銆�

7.閻佃泲鐧斤細澶氭暩(sh霉)鎮ｈ€呴惖铔嬬櫧鏄庨’鍗囬珮锛岃┎闋呮鏌ヨ垏鐤剧梾 鐨勮綁(zhu菐n)姝稿瘑鍒囩浉闂�(gu膩n)锛屽彲浣滅偤妾㈡脯鑷ㄥ簥鐧傛晥鐨勬寚妯�(bi膩o)銆�

8.绱�(x矛)鑳炴瘨鍔熻兘瀛�(xu茅)妾㈡煡锛氬寘鎷� NK 绱�(x矛)鑳炲姛鑳�銆丆D107a銆佺┛瀛旂礌銆侀绮掗叾銆丮unc13-4 绛�锛屾寔绾�(x霉)鎬� NK 绱�(x矛)鑳炲姛鑳芥槑椤笅闄嶏紝鍜�(鎴�)娴佸紡绱�(x矛)鑳炲(xu茅)妾㈡煡 NK/CTL 绱�(x矛)鑳炶〃闈笂杩拌泲鐧借〃閬�(d谩)姘� 骞充笅闄�锛屾噳(y墨ng)娉ㄦ剰 FHLH 鐨勫彲鑳芥€�銆�

9.鑵归儴B瓒咃細鍙槑纰鸿倽銆佽劸銆佽吂鑵旀穻宸寸祼(ji茅)鑵ぇ鎯呮硜锛屽悓鏅傛帰鏌ユ湁鐒¤嚐鍣ㄥ璩�(zh矛)鐣板父鍙婂悇绋崰浣嶆€х梾璁�锛屽湪鍔╄êHLH鐨勫熀绀�(ch菙)涓婇€�(j矛n)涓€姝ュ畬鍠勭梾鍥犺ê鏂�銆�

10.鑳搁儴CT锛氳偤閮ㄥ彈绱殑鎮ｅ厭鍙〃鐝�(xi脿n)鐐洪枔璩�(zh矛)鎬ц偤鐐庯紝閲嶈€� 涔熷彲鏈夋枒鐗囩媭鎴栧ぇ鐗囧奖绛夎偤瀵﹁唱(zh矛)鍙楃疮鏀硅畩鍙婅兏鑵旂⿳娑茬瓑琛ㄧ従(xi脿n)銆�

11.闋儴MRI锛氫腑妯炵缍�(j墨ng)绯荤当(t菕ng)鍚勫€嬮儴浣嶅潎鍙彈绱�锛屾棭鏈熷琛ㄧ従(xi脿n)鐐鸿叇婧濆洖澧炴繁銆佸瀵瓑杌熻叇鑶滃彈绱緛璞�锛屼富瑕佺偤娣嬪反绱�(x矛)鑳炲強宸ㄥ櫖绱�(x矛)鑳炴蹈娼ゆ墍鑷�锛屾澶栭倓鍙鑵﹀鎿�(ku貌)寮电瓑鍚勭ó鑵﹁悗绺ǎ鏀硅畩;涔熷彲鏈夎叇鐧借唱(zh矛)鑴珦闉樺強澹炴绛夎〃鐝�(xi脿n)銆�

12.鑵﹁剨娑�(CSF)妾㈡煡锛氬鐥呬汉鐥呮儏鍏佽ū锛孒LH鎮ｅ厭鍧囨噳(y墨ng)閫�(j矛n)琛岃叇鑴婃恫妾㈡煡锛屽鍚堝苟涓鍙楃疮锛孋SF涓窗(x矛)鑳炴暩(sh霉)鎴栬泲鐧藉崌楂�锛岀窗(x矛)鑳炰互娣嬪反绱�(x矛)鑳炲崌楂樼偤涓伙紝鍙湁鍠牳绱�(x矛)鑳�锛屽皯閮ㄥ垎鎮ｅ厭鍙鍣绱�(x矛)鑳�銆傝叇鑴婃恫鐣板父鏀硅畩鏄疕LH闋�(y霉)鍚庝笉鑹殑閲嶈鍥犵礌銆�

13.鐥呭師瀛�(xu茅)妾㈡煡锛氱敤浜庨憭鍒ユ劅鏌撳洜绱犲皫(d菐o)鑷寸殑HLH锛屽寘鎷珽BV銆丆MV銆丠SV銆丠HV-6銆丠HV-8銆佽吅鐥呮瘨鍜屽井灏忕梾姣払19绛夋姉楂擠NA鐨勬娓�锛屼互鍙婃敮鍘熼珨銆佺祼(ji茅)鏍�銆佸竷姘忔】鑿�銆侀粦鐔辩梾绛夌浉闂�(gu膩n) 妾㈡脯

14.HLH鐩搁棞(gu膩n)鎬у熀鍥犳鏌ワ細宸茬櫦(f膩)鐝�(xi脿n)绱�20浣欑ó鍩哄洜缂洪櫡鑸囧師鐧�(f膩)鎬LH鐨勭櫦(f膩)鐥呭瘑鍒囩浉闂�(gu膩n)锛屽彲閫氶亷鍩哄洜娓簭鐨勬柟娉曚簣浠ョ簿纰烘脯瀹氾紝鍏烽珨鍩哄洜鍖呮嫭PRF1, UNCl3D, STX11, STXBP2, RAB27A, LYST, SH2D1A, BIRC4, ITK, AP3B1, MAGT1, CD27绛�?锛涱媰椹抸鏌ョ櫦(f膩)鐝�(xi脿n)鏈夌獊璁�锛屾噳(y墨ng)瑭茬祼(ji茅)鍚圢K娲绘€с€丆D107a琛ㄩ仈(d谩)绛夊姛鑳借│椹楃祼(ji茅)鍚堢稖鍚堝垽鏂�銆�

15.鍏朵粬锛氬鏁�(sh霉)鎮ｈ€匧DH鏄庨’澧為珮锛屾澶栬厧鑷熷彈绱彲鏈夎灏�銆佽泲鐧藉翱锛岄噸鑰呭彲鏈夋爱璩�(zh矛)琛€鐧�;鑵﹀璩�(zh矛)鍙楃疮鏅傝叇闆诲湒妾㈡脯鍙湁鐣板父鏀硅畩銆�

(涓�)鍏掔HLH鐨勮ê鏂锋(bi膩o)婧�(zh菙n)

鐩墠浠嶅弮鐓у湅闅涚祫绻旂窗(x矛)鑳炲崝(xi茅)鏈�2004 骞村埗瀹氱殑瑷烘柗妯�(bi膩o)婧�(zh菙n)銆�

(鍥�)閼掑垾瑷烘柗

HLH鐨勮ê鏂峰苟涓嶅洶闆�锛岀敱浜庢不鐧傛柟娉曚笉鍚�锛岄憭鍒ュ紩璧稨LH鐨勫師鍥犻潪甯搁噸瑕�锛宲HLH涓昏閫氶亷鍩哄洜?q奴)W妾㈡脯鑸噑HLH鐩稿崁(q奴)鍒ャ€俿HLH涓昏鍖呮嫭鎰熸煋鐩搁棞(gu膩n)HLH銆侀ⅷ(f膿ng)婵曞厤鐤€х柧鐥呯浉闂�(gu膩n)HLH浠ュ強鑵槫鐩搁棞(gu膩n)HLH銆�

1.鎰熸煋鐩搁棞(gu膩n) HLH锛氫互鐥呮瘨鎰熸煋鏈€甯歌锛屼富瑕佽浜庣毎鐤圭梾姣掍腑鐨� EBV 鎰熸煋锛屽叾浠栫梾姣掑 CMV銆佸叾瀹冪毎鐤圭梾姣�銆佹祦鎰熺梾姣掔瓑锛屽叾浠栫梾鍘燂紝濡傜祼(ji茅)鏍告】鑿�銆佸竷姘忔】鑿屻€佹敮鍘熼珨銆佹潨姘忓埄浠€鏇煎師锜茬瓑鍧囧彲灏�(d菐o)鑷� HLH 鐧�(f膩)鐢�銆傛劅鏌撶浉闂�(gu膩n) HLH 涓昏闈犵梾鍘熷(xu茅)瑷烘柗浠ラ憭鍒�銆�

2.绻肩櫦(f膩)浜庨ⅷ(f膿ng)婵曞厤鐤€х柧鐥呯殑 HLH锛氬張绋卞法鍣窗(x矛)鑳炴椿鍖栫稖鍚堝緛(macrophage activation syndrome, MAS)銆傛渶甯歌浜庡叏韬瀷骞煎勾鐗圭櫦(f膩)鎬ч棞(gu膩n)绡€(ji茅)鐐�锛屼篃甯歌浜庡叾浠栭ⅷ(f膿ng)婵曞厤鐤€х柧鐥�锛岃┎椤炵柧鐥呰垏鍏朵粬 HLH 鐨勪富瑕佸崁(q奴)鍒ユ槸鏈夐ⅷ(f膿ng)婵曞厤鐤€х柧鐥呯殑鐩搁棞(gu膩n)琛ㄧ従(xi脿n)锛屽鐧�(f膩)鐔变即鐨柟銆侀棞(gu膩n)绡€(ji茅)鐐庛€佽嚜韬姉楂旀淮搴﹀崌楂樼瓑銆�

3.鑵槫鐩搁棞(gu膩n) HLH锛氬绻肩櫦(f膩)浜庤娑茬郴绲�(t菕ng)鎯℃€ц叓鐦�锛屽湪鍏掔甯歌绻肩櫦(f膩)浜庢穻宸寸槫(灏ゅ叾鏄枔璁婃€уぇ绱�(x矛)鑳炴穻宸寸槫鎴� NK/T 绱�(x矛) 鑳炴穻宸寸槫)銆佺櫧琛€鐥�(澶氳浜� T 绱�(x矛)鑳炲瀷)銆傛湕鏍肩綍绱�(x矛)鑳炵祫绻旂窗(x矛) 鑳炲鐢熺櫏鎮ｅ厭涔熷彲骞剁櫦(f膩) HLH銆傜梾鐞嗚ê鏂锋槸閼掑垾鐨勯棞(gu膩n)閸�銆�

鍥�銆佹不鐧�

(涓€)鏃╂湡娌荤檪

HLH 鐥呮儏鍏囬毆锛岄€�(j矛n)灞曡繀閫熴€備笉鍙婃檪娌荤檪鍏剁敓瀛樻檪闁撳緢灏戣秴閬� 2 鍊嬫湀锛屾墍浠ユ棭鏈�銆佹伆鐣�(d膩ng)鍜屾湁鏁堢殑娌荤檪闈炲父閲嶈銆傜枒瑷篐LH锛岄渶鐩″揩(24锝�48 灏忔檪鍏�(n猫i))瀹屾垚鎵€鏈� HLH 纰鸿ê妾㈡煡鍙婄浉闂�(gu膩n)鐥呭洜?q奴)W妾㈡煡锛屼竴鏃︾鍚堣ê鏂锋(bi膩o)婧�(zh菙n)锛屾噳(y墨ng)绔嬪嵆闁嬪娌荤檪銆�

(浜�)鍒嗗堡娌荤檪 HLH鏄竴椤炵稖鍚堝緛锛屽彲鐢卞绋師鍥犲紩璧凤紝娌荤檪鎳�(y墨ng)鐩稿皪鍊嬮珨鍖�锛屽苟闈炴墍鏈夋偅鑰呭潎鍤�(y谩n)鏍兼寜鐓LH-1994鏂规瀹屾垚鍏ㄩ儴鐧傜▼锛屽皪涓€浜涜純杓曠殑HLH鎮ｈ€�(鍖呮嫭pHLH)鍠敤婵€绱犲彲鑳芥帶鍒剁梾鎯�銆傛不鐧傞亷绋嬩腑鎳�(y墨ng)瀵嗗垏瑙€瀵熺梾鎯呰畩鍖�锛屾湁姊濅欢鐨勫柈浣�锛屽彲浠ョ洠(ji膩n)娓窗(x矛)鑳炲洜瀛愯瓬锛岄毃鏅傝⿻浼�锛屾牴鎿�(j霉)鑷ㄥ簥琛ㄧ従(xi脿n)銆佺窗(x矛)鑳炲洜瀛愯瓬璁婂寲鎯呮硜绛夎⿻浼扮祼(ji茅)鏋滃強鏅傝(di脿o)鏁存不鐧傛柟妗�銆傚皪浜庨洠娌诲京(f霉)鐧�(f膩)鐥呬汉鍦ㄦ不鐧傞亷绋嬩腑浠嶉渶涓嶆柗鏌ユ壘鍘熺櫦(f膩)鐥�銆�

1.鍘熺櫦(f膩)鐥呯殑娌荤檪锛氭牴鎿�(j霉)寮曡捣HLH鐨勪笉鍚屽師鐧�(f膩)鐥呯郸浜堢浉鎳�(y墨ng)娌荤檪銆�

2.鍖栫檪锛氱洰鍓嶄互鍦嬮殯绲勭箶绱�(x矛)鑳炲崝(xi茅)鏈冪殑HLH-1994鏂规鐐哄熀绀�(ch菙)锛屼富瑕佸寘鎷冻钁変箼鐢欍€佺硸鐨唱(zh矛)婵€绱犲拰鐠�(hu谩n)瀛㈢礌銆�

(1)瑾樺皫(d菐o)娌荤檪(8鍛�)

鐢叉綉灏奸緧(MP)锛氶潨鑴堟淮娉紝10 mg/(kg· d)×3 d锛� 5 mg/(kg· d)×3 d锛� 2 mg/(kg· d)×8 d锛�1 mg/(kg· d) ×2鍛紝0.5 mg/(kg· d)×2鍛�锛�0.25 mg/(kg· d)×1鍛�锛岀辜浜�1鍛ㄥ収(n猫i)娓涘仠锛岀檪绋嬪叡8鍛�銆俈P-16锛氶潨鑴堟淮娉�锛�100 mg/(m²·娆�)锛�2娆�/鍛�×1鍛�锛�1 娆�/鍛�×7鍛�銆侰SA锛氬彛鏈�锛�5 mg/(kg· d)锛屽垎2娆★紝姣�12灏忔檪1娆�锛岃嚜 鍖栫檪绗�15澶╄捣銆傝钘ユ績搴�(璋锋績搴�)涓嶈秴閬�200 μg/L銆傞灅娉細鍖栫檪鍓�(鎮ｅ厭鍑哄嚌琛€鍔熻兘鍏佽ū鐨勬儏娉佷笅)鍜屽寲鐧�2鍛ㄦ檪(鍖栫檪鍓岰SF鐣板父)琛岃叞绌�锛屽2鍛ㄥ悗涓绁炵稉(j墨ng)绯荤当(t菕ng)鐧ョ媭鍔犻噸鎴朇SF鐣板父鐒℃敼鍠�(鍖呮嫭绱�(x矛)鑳炴暩(sh霉)鍜岃泲鐧界檪锛屾瘡鍛�1娆�锛屽叡4鍛�銆� 娉細瑾樺皫(d菐o)娌荤檪閬庣▼涓渶姣�1锝�2鍛ㄨ⿻浼扮梾鎯呭強HLH瑷烘柗鐩搁棞(gu膩n)鎸囨(bi膩o)銆�

(2)缍寔娌荤檪(9锝�40鍛�)

闄ゅpHLH鍜孧AS锛岀8鍛ㄨ⿻ 浼癈R鑰呬笉闇€瑕佺辜绾�(x霉)缍寔娌荤檪锛岀董鎸佹不鐧傜殑鐩殑鏄偤浜嗛渶瑕佺Щ妞嶇殑鐥呬汉绛夊緟閫犺骞茬窗(x矛)鑳炵Щ妞�锛岃ê鏂稭AS鐨勭梾浜哄彲鎸夌収鐩告噳(y墨ng) 鐨勭柧鐥呴€�(j矛n)琛岀董鎸佹不鐧�銆侱ex锛氬彛鏈�锛�10 mg/(m²·d)×3 d姣�2锛屽懆1娆�锛岀9鍛ㄨ捣銆俈P-16锛氶潨鑴堟淮娉�锛�100 mg/(m²·娆�)锛屾瘡2鍛�1娆★紝绗�10 鍛ㄨ捣銆� CSA锛氱辜绾�(x霉)鍙ｆ湇锛岃钘ユ績搴�(璋锋績搴�)涓嶈秴閬�200 μg/L銆傛敞锛氱董鎸佹不鐧備腑闇€瑕佹瘡4鍛ㄨ⿻浼癏LH瑷烘柗鐩搁棞(gu膩n)鎸囨(bi膩o)锛屽皪浜庣辜鐧�(f膩)HLH(闄ゅMAS)锛岀梾鎯呭畬鍏ㄧ珐瑙ｅ彲鍋滄HLH鐩搁棞(gu膩n)鍖栫檪銆備絾濡傚仠钘ュ悗鍑虹従(xi脿n)HLH寰�(f霉)鐧�(f膩)锛屽墖鎳�(y墨ng)鍙婃檪鎺у埗鐥呮儏鍚庣洝鏃╅枊灞曢€犺骞茬窗(x矛)鑳炵Щ妞嶆不鐧�銆�

3.鏀寔娌荤檪锛氬強鏅傝檿鐞嗗嚭琛€銆佹劅鏌撳拰澶氳嚐鍣ㄥ姛鑳借“绔瓑 骞剁櫦(f膩)鐧ユ槸闄嶄綆姝讳骸鐜囩殑閲嶈鍥犵礌銆� 4.閫犺骞茬窗(x矛)鑳炵Щ妞嶏細灏嶄簬pHLH銆佸弽寰�(f霉)寰�(f霉)鐧�(f膩)鎴栬€呯稉(j墨ng)涓€绶氬拰浜岀窔娌荤檪鏁堟灉涓嶄匠鐨勯洠娌绘€LH鎮ｅ厭鎳�(y墨ng)鐩℃棭鎺ュ彈閫犺骞茬窗(x矛)鑳炵Щ妞�銆�

浜�銆佺柧鐥呯媭鎱�(t脿i)鐨勫畾缇�

(涓€)鑷ㄥ簥鍙嶆噳(y墨ng)(Clinical response)

婊胯冻浠ヤ笅5鍊嬫浠讹紝鐢ㄤ簬瑾樺皫(d菐o)娌荤檪鏈�锛屽垽鏂锋槸鍚︽寜瑭叉柟妗堢辜绾�(x霉)閫�(j矛n)琛屽寲鐧�銆�1.鐒＄櫦(f膩)鐔�;2.鑴捐嚐绺皬;3.琛€灏忔澘>100×10⁹/L;4.绾栫董铔嬬櫧鍘熸甯�;5.閻佃泲鐧戒笅闄�>25%銆�

(浜�)鐤剧梾鐒℃椿鍕曟垨瀹屽叏绶╄В(Non-active disease or resolution)

鐢ㄤ簬鍒ゆ柗8鍛ㄨ獦灏�(d菐o)娌荤檪鍚庢槸鍚﹂渶瑕佺董鎸佹不鐧�銆�1.鐒＄櫦(f膩)鐔�;2.鐒¤劸鑵ぇ(閮ㄥ垎鐥呬汉鍙柈鐛�(d煤)瀛樺湪涓害鑴捐叓澶�);3.娌掓湁琛€绱�(x矛)鑳炴笡浣�(琛€绱呰泲鐧�>90 g/L锛岃灏忔澘>100 ×10⁹/L锛屼腑鎬х矑绱�(x矛)鑳�>1×10⁹/L);4.鐢樻补涓夐叝姝ｅ父;5.閻佃泲鐧�<500μg/L;6.鑵﹁剨娑叉甯�(灏嶄簬鐥呭垵鑵﹁剨娑蹭笉姝ｅ父鐨勬偅鍏�);7.鍙憾鎬D25姝ｅ父銆�

(涓�)鐤剧梾娲诲嫊(Active disease)

娌荤檪鍚庢湭閬�(d谩)鍒颁笂杩扮柧鐥呯劇娲诲嫊姊濅欢鐨勭梾浜�銆�

(鍥�)鐤剧梾鍐嶆縺娲�(Reactivation of disease)

宸查仈(d谩)鍒板畬鍏ㄧ珐瑙�锛屽張鍑虹従(xi脿n)浠ヤ笅8姊濅腑鐨�3姊濆強浠ヤ笂鐨勭梾浜恒€�1.鐧�(f膩)鐔�;2.鑴捐叓澶�; 3.琛€灏忔澘<100×10⁹/L;4.鐢樻补涓夐叝>3 mmol/L;5.绾栫董铔嬬櫧鍘�<1.5 g/L;6.楠ㄩ珦鐧�(f膩)鐝�(xi脿n)鍣鐝�(xi脿n)璞�;7.閻佃泲鐧�>500μg/L;8.鍙憾鎬D25>2400 U/L銆傛敞锛氬鏋滃嚭鐝�(xi脿n)鏂扮殑CNS鐧ョ媭(闄ゅ鍏朵粬鐤剧梾)渚垮彲瑷烘柗鍐嶆縺娲�銆�

鍏�銆侀毃瑷�

(涓€)鍋滆棩鍚�1骞村収(n猫i)姣� 3 鍊嬫湀宸﹀彸琛�1娆¤甯歌(gu墨)銆佺敓鍖�銆佸嚌琛€銆乻CD25銆侀惖铔嬬櫧銆丒BV 妾㈡脯(灏嶄簬 EBV-HLH)銆佺窗(x矛)鑳炴瘨鍔熻兘(鐥呭垵鐣板父鑰呭京(f霉)鏌�)銆侀牳閮ㄥ拰鑵归儴B瓒呮鏌�锛屾湁涓鍙楃疮鎮ｅ厭姣� 6 鍊嬫湀寰�(f霉)鏌ラ牠椤� MRI銆�

(浜�)鍋滆棩 1 骞翠互鍚庢瘡骞磋1娆¤甯歌(gu墨)銆佺敓鍖栨鏌ュ拰EBV妾㈡脯(灏嶄簬EBV-HLH)锛屽苟琛屽父瑕�(gu墨)鍏掔楂旀牸妾㈡煡銆傚嚭鐝�(xi脿n)寰�(f霉)鐧�(f膩)鐧ョ媭闅ㄦ檪寰�(f霉)瑷�銆�

涓�銆佽綁(zhu菐n)瑷烘浠�

(涓€)寰炰笂绱氶啱(y墨)闄㈣綁(zhu菐n)瑷哄埌涓嬬礆鎴栧熀灞ら啱(y墨)闄�

1.HLH瑷烘柗鏄庣⒑锛孒LH鍘熺櫦(f膩)鐥呮槑纰�;

2.HLH鐥呮儏鐩稿皪绌�(w臎n)瀹�锛岃嚚搴婃矑鏈夋椿鍕曟€у嚭琛€鍙婅嚐鍣ㄥ姛鑳借“绔〃鐝�(xi脿n);

3.娌荤檪鏂规纰哄畾涓斿悗绾�(x霉)娌荤檪鍙互鍦ㄧ暥(d膩ng)?sh霉)钂痶(y墨)闄㈠畬鎴�銆�

(浜�)寰炲熀灞ら啱(y墨)闄㈣綁(zhu菐n)瑷哄埌涓婄礆閱�(y墨)闄�

1.鎿êHLH浣嗙劇娉曟槑纰鸿ê鏂锋垨鐥呭洜涓嶆槑;

2.鏂拌ê鏂稨LH锛岀暥(d膩ng)?sh霉)钂痶(y墨)闄㈢劇娌荤檪姊濅欢;

3.闆ｆ不HLH鐥呬汉锛屼竴绶氭柟妗堟不鐧傜劇娉曟帶鍒剁梾鎯�;

4.闆ｆ不寰�(f霉)鐧�(f膩)鎴栧師鐧�(f膩)HLH鐥呬汉鐣�(d膩ng)?sh霉)璨�(z茅)o閫犺骞茬窗(x矛)鑳炵Щ妞嶆浠�