被套區細胞淋巴瘤—兼述非何杰金氏淋巴瘤的新進分類

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ၡāāࢋ

( Mantle cell lymphoma MCL ) ( Non-Hodgkin's lymphoma )

( B

) MCL Cyclin D1 11 14

t ( 11 14 ) B CD19 CD20 CD22 CD23 CD10

( CALLA ) T CD⁵

58 ( 71%-90% ) ( 35%-55% ) (53%-

90% )

( MIPI ) MCL

(

) ( 47% VS 49% )

ᙯᔣෟĈజइડ௟ࡪ୽͐ሳ ( Mantle cell lymphoma, MCL )

ܧңڔܛͩ୽͐ሳ ( Non-Hodgkin's lymphoma ) ۞̶ᙷ

ܧңڔܛͩ୽͐ሳ۞ঽநኑᗔّΞᄲߏ඗൑

่ѣĂЯࠎι͉кᇹ̼˞Ąι۞ঽந̶ᙷ౵ѝ৷ Ϥݭၗ̶ᙷĂ҃ѣ୽͐ሳ ( Lymphoma )ă୽͐҇

܂ሳ ( Lymphosarcoma ) ͽ̈́შې௟ࡪ҇܂ሳ ( Reticulum cell sarcoma ) ˬᙷĂன̏ୢ̝̙ϡĄ д 1966 ѐĂ Henry Rappaport ͽ௟ࡪ ( ̂̈ ) ̈́௡

ᖐ ( ඕ༼ّ nodular ٕᚢႝّ diffuse ) ̶ֽᙷ҃஽

Җٺ̱ă˛˩ѐ΃۞࡚߷Ą

ለ߷݋ѣ Luke & Collins ( 1974 ) ̈́ Kiel ( 1975

̈́ 1988 Լ։ ) ۞̶ᙷڱĂҭͧྵ݀ࢨّĄТॡഇ ᔘѣ W H O ă D o f m a n ̈́ࡻ઼۞୽̶͐ᙷڱ¹ ( B r i t i s h N a t i o n a l Ly m p h o m a I n v e s t i g a t i o n Classification )ĄϤٺ̶͉ڡ˞Ă҃ѣ઼઼࡚छᒛ

া ࡁ տ ੰ ۞ ̍ ү ̶ ᙷ ² ( N C I W o r k i n g FormulationĂ 1982 )Ăι౵ࢦࢋ۞ಶߏ૟୽͐ሳ

̶ࠎҲޘă̚ޘ̈́੼ޘೋّˬ̂ᙷĂ҃ࢲҖٺˣ

˩̈́˝˩ѐ΃Ąז˞ 1994 ѐĂ઼ᅫ୽͐ሳࡁտ

௡૟Һࠪ௟ࡪ̼ጯᑕϡٺѩ҃૟׎̶ࠎ B ̈́ T ௟ ࡪ׌̂ᙷĂӈٙᏜ۞Լ։ّለ࡚ܧᙷڱ³( REAL )Ą д 1999 ѐĂ͵ࠧ኎Ϡ௡ᖐ ( WHO ) ᄮТ֭Լซ ( Β߁Հкᙷ )Ă҃ѣன̶̫̝ᙷ⁴Ąιͽ '݈ B'Ă 'B'Ă'݈ T' ᄃ 'T' ௟ࡪͽ̈́ңڔܛͩ୽͐ሳඈ̣̂

ᙷޢГ௟̶Ą

జइડ௟ࡪ୽͐ሳ׻Щ۞ֽ໚

"జइડ௟ࡪ୽͐ሳ" ( Mantle cell lym- phomaĂͽ˭ᖎჍ MCL ) ߏд˝˩ѐ΃ѝഇĂ ࠎ Raffeld, Jaffe⁵̈́ Banks⁶ٙϒё೩΍Ą΁ࣇೡ

ࢗࣧд̈୽͐௟ࡪ୽͐ሳ۞পঅֲݭĄιࣇѣ

፾প۞௟ࡪߖҒវளҜ ( Translocation ) ጱ࡭

BCL1 ( B௟ࡪ୽͐ሳ1 ) ۞ૄЯள૱Ă҃ѣకϨ ኳ C y c l i n D¹۞࿅ޘயϠĄѝд 1 9 8 2 ѐĂ We i s e n b u r g e r⁷̈́ P a l u t k e⁸അೡࢗдᕭڽّ

( Follicular ) ୽͐ሳ̰۞পঅ௡ཏĂιࣇѣܧ׏

ݭ̈୽͐஧ಛᖒд։ّᆧത ( Germinal ) ͕̚γ

̝జइડ ( ဦ˘ )Ą΁ࣇ࠹ܫ఺ߏᚢႝّ̚ޘ̶

̼୽͐஧୽͐ሳ ( Diffuse intermediate lympho- cytic lymphoma - Rappaport̶ᙷڱ ) ۞ᕭڽ၆໰

௡ ( Follicular Counterpart )Ă҃Ⴭ̝ࠎ "జइડ"

( Mantle Zone ) ୽͐ሳĄ

д˝˩ѐ΃ѝഇᙋځιࣇߏᚢႝّ̈ෘ८௟

ࡪ୽͐ሳ ( Diffuse - small cleaved cell lymphoma )

̈́̈୽͐௟ࡪ୽͐ሳ ( small lymphocytic lym- phoma )۞ֲݭĄι۞ݭၗᔵᄃ˯ࢗ׌჌୽͐ሳ ( ቤၙϠܜݭ )ᙷҬĂҭߏ࿰ޢݒߏܧ૱̙р۞˘

ཏĄঽந࠻Ҭ̙֭ިᆃೋّ ( low grade )ĂҭᓜԖ

ܑனݒည̚ޘೋّĂΞਕд 2 - 3 ѐνΠಶ࿅͵

( Ϗགྷڼᒚ )Ąҭ၆ڼᒚ۞ͅᑕ˫ညҲޘೋّĂ˵

ಶߏܧ૱۞̙நຐĂΞͽᄲд୽͐ሳ̚౵ᙱڼᒚ

۞˘჌Ąѩޢ޵Һࠪ௡ᖐߖҒ̈́າ۞௟ࡪߖҒវ

( cytogenetic immunohistochemistry )Ăͽ̶̈́̄Ϡ ۏጯ ( Molecular biology ) ̝ኲĂ Raffeld ඈˠ଀ͽ

૟׎ᕩࠎາ۞జइડ௟ࡪ୽͐ሳĄ

జइડ௟ࡪ୽͐ሳ۞ᓜԖܑன

M C Lд˯ࢗ۞̶ᙷᔵᛳٺҲޘೋّĂҭ၁ ᅫ˯˫ତܕ̚ޘೋّĂࡗҫٙѣܧңڔܛͩ୽͐

ሳ۞ 5%-8% Ąр൴ٺշّ ( շĈ̃ࠎ 2 Ĉ 1 )⁹Ă

̚ม൴ঽѐ᛬ࡗࠎ 5 8 ໐Ăд෧ᕝॡ૱̏ߏ୎

ഇĄ˩ѐх߿தΪѣ 8% ( ࠹၆ٺ׎΁ܧңڔܛ

ͩ୽͐ሳ۞ 35% )Ą̂ొ̶۞ঽଈͽБ֗୽͐ඕ ཚܑ̂னĂ૱ѣքත۞ཚ̂âΗͽ˯ѣ੻ល۞

ܬϚĂΩγೀͼౌѣབࡤ྽۞ܬϚĂ׎΁ጡء݋

Β߁۱̈́హొ௡ᖐĄΩγѣα̶̝˘۞ঽˠѣᒛ

௟ࡪ΍னдฉᙝҕ୵ ( ဦα )Ăѩ჌ঽˠѣ 6% ۞ ဦ˘Ĉ୽͐ሳд୽͐ඕ̰۞௟ࡪ໚੓఍Ą

B-௟ࡪ୽͐ሳ T-௟ࡪ୽͐ሳ

ᕭڽّ୽͐ሳ ұᒑপ୽͐ሳ

జइડ௟ࡪ୽͐ሳ

ᙝቡડ୽͐ሳ

Ϗ̶̼̂௟

ࡪ୽͐ሳ

̈ B-௟ࡪ

ၙّ୽͐஧Ϩҕঽ

̈୽͐஧୽͐ሳ λ஧కϨҕঽ

׹ᙝ T-௟ࡪ୽͐ሳ ϩܑ T-௟ࡪ୽͐ሳ

ොċ F,ᕭڽĒMC,លኳ৶ĒPC,य़ϩኳĒS,ᚭ

፟ົܬϚז̚ᇽৠགྷ¹⁰Ą

M C L۞̶ഇڻϡ˘ਠңڔܛͩ୽͐ሳ۞̶

ഇڱĂӈ Ann-Arbor ̶ഇڱ ( I-IV ̈́ A ٕ B )Ă఺

༊൒ᄃι۞࿰ޢѣᙯĄѩγĂܧңڔܛͩ୽͐ሳ ᔘѣ࿰ޢ޽ᇴ ( International Prognostic Index Ăᖎ Ⴭ IPI )¹¹ĂϤѐ᛬ ( ŵ 60 ໐ )ăវ˧ ( Performance statusĂ ECOG Ÿ 2 )ăഇҾ ( ǷٕǸ )ă୽͐ඕγ ܬϚ ( Ÿ 2 )Ăͽ̈́ LDH ( Ÿϒ૱˯ࢨ )ֽග̶Ă ߁ཱི̰۰Чࠎ˘̶Ă̶ᇴ෸੼۰࿰ޢ෸मĄҭд M C L ݋ྵዋϡᕭڽّ୽͐ሳ۞઼ᅫ࿰ޢ޽ᇾ ( Follicular Lymphoma International Prognostic IndexĂᖎჍ FLIPI )^12,13Ăιࣇ۞मளࠎ FLIPI ̙ Еˢវਕ̈́୽͐ඕγ۞ܬϚᇴĂҭΐˢ୽͐ඕડ

۞ܬϚᇴ ( Ÿ 5 )Ă̈́ѣ൑ళҕ ( Hb Ŵ 12 g/dl )Ą M o l l e r ඈ۞ࡁտĂͧྵ˯ࢗ׌჌޽ᇴܑனĂ FLIPIΞͽ࿰ീ MCL ۞ϠхதĂҭ IPI ݋̙ਕĄ Яѩޙᛉϡ FLIPI үࠎ MCL ۞࿰ޢ޽ᇾ¹⁴Ą౵ܕ Hosterඈ΃ܑᇇ઼̈́ለ߷୽͐ሳࡁտშ̶ژ 455

ּ MCL¹⁵Ăᄮࠎ˯ࢗ׌჌޽ᇴ࠰ѣ̙֖̝఍Ă҃

Ωޙϲ૞၆ MCL ۞઼ᅫ࿰ޢ޽ᇾჍࠎ MIPI Ą΁

ࣇ̪ଳϡѐ᛬Ăវ˧̈́ L D H Ăҭΐˢ W B C ᇴ ( ͽ 10X10⁹ࠎ̶ࠧ )ć҃૟ MCL ̶ࠎҲĂ̈́̚੼

ˬПᐍཏĄ఺၆׎࿰ޢ۞࿰ീٕధՀࠎѣϡĄ

జइડ௟ࡪ୽͐ሳ۞ঽந෧ᕝ

M C L۞ঽநѣॡटٽజᄱ෧ࠎ΁჌۞୽͐

ሳĂ͍׎ߏ੊ቚٕགྷរ̙֖۞ᗁरĄ఺ొ̶ࣧЯ ߏιд˝˩ѐ΃ޢ̶̖΍۞পள୽͐ሳĄ Fisher ඈ̶ژ SWOG ( ઼࡚Ҙݑᒛাࡁտཏ௡ )ೀ࣎ᓜ

Ԗྏរ۞ঽˠĂВ 376 ּ ( 1972-1983 )¹⁴Ąඕڍ൴ னѣ 10% ᕩᙷд׎΁୽͐ሳ ( ܑ˘¹⁴)Ą͍׎ߏд EᙷĂӈᚢႝݭ̈ෘ८௟ࡪ ( Diffuse small cleaved )

̶ٕ̼̙։ ( Diffuse Poorly differentiated )̚ܕˬ

̶̝˘ࠎ MCL¹⁵Ą

ѝഇ۞ MCL Ăѣ 30% Ӕඕ༼ݭ ( Nodular Ă ဦ˟˯ )Ă׎ඕ༼ѣܧ׏ݭ̈୽͐஧۞ওማĄѣ

ֱඕ༼Ϥᕭڽ̈́ͅᑕّᆧത͕̚ ( G e r m i n a l Center ) ᆧϠĂ׹ಛѣ̈୽͐஧ӔజइېᕖૺĂ

Я҃଀Щ^{7 , 1 6}Ą༊׎дซҖॡĂາϠ۞ᒛ௟ࡪও

ማזᕭڽม۞г૲Ăֹ଀ඕ༼ّ႙႙ঐεĂซ҃

ѣ ඕ ༼ ̈́ ᚢ ႝ ݭ ۞ Ϡ ܜ Ă ౵ ௣ ݋ ј ࠎ ᚢ ႝ ّ ( Diffuse MCLĂဦ˟˭̈́ဦˬ )Ą݈۰ ( Nodular )۞

˩ѐх߿தࠎ 14% Ă҃ޢ۰ ( Diffuse ) ݋ࠎ 10% Ą ᔘѣ˘჌܂஧តள჌ ( Blastoid variant Ăဦα ) ݋ࠎ

࿬Ą౵ޢ఺჌Ϗ̶̼ݭѣྵк۞௟ࡪ̶ෘĂ௟ࡪ

ܑ˘Ĉజइડ௟ࡪ୽͐ሳд 1972-1983 ̝ࣧ୽͐ሳ෧ᕝ ( SWOGྤफ़ )

ࣧ෧ᕝ ᓁᇴ NDMѺ̶ͧ

A ඕ༼ّ୽͐௟ࡪᄃ௡ᖐ௟ࡪݭ୽͐ሳ 40 0 0

B ඕ༼ّ௡ᖐ௟ࡪݭ୽͐ሳ 29 0 0

C ඕ༼̶ّ̼̙։୽͐ሳ 171 9 5%

D̶̼։р୽͐௟ࡪݭ୽͐ሳ 70 6 9%

E ᚢႝ̶ّ̼̙։୽͐ሳ 66 21 32%

ኑᑭᓁᇴ 376 36 10%

ොĈ SWOG Ă઼࡚Ҙݑᒛাࡁտཏ௡ć MCL Ăజइ ડ௟ࡪ୽͐ሳ

ဦ˟Ĉజइડ௟ࡪ୽͐ሳ۞୽͐ඕঽந̷ͯĂ H & E ߖ ҒĂҲࢺ( 1 0 X 4 ) Ą˯ဦពϯඕ༼ّĂ˭ဦពϯ ᚢႝّĄ

ྵ̂Ă८̥ྵځពĂ८ߖҒវ̶೸௟ቜ¹⁷Ą

జइડ௟ࡪ୽͐ሳ۞ϠۏপّůҺ

ّࠪ̈́ૄЯপّ

Ϥٺಏያݭၗֽ෧ᕝ MCL ѣॡົѣӧᙱĂ ЯѩҺࠪ௟ࡪ̼ጯᑭߤٕ۰Һࠪ௡ᖐ̼ጯᑭߤಶ ត଀ޝࢦࢋֽᑒӄ෧ᕝ ( ܑ˟^9,20)ĄࢵАߏ Cyclin D¹υࠎวّ¹⁸ ( ѣಡӘࠎౚّ۰Ăҭ׎ Cyclin D² ࠎวّ¹⁹ )Ą׏ݭ۞ MCL ѣߖҒវ 11 ̈́ 14 ۞ள ҜĪt ( 11 Ă 14 ) q13 Ă q32īĂ BCL1ૄЯдѩள ҜĪιߏ஧కϨࢦᙥ۞ܳј۰ ( Promoter )īĂጱ

࡭ Cyclin D¹۞ᆧϠ҃ҺࠪߖҒࠎวّĄ׎Ѩߏ ھ B ( Pan B ) ௟ࡪ۞ܑᇈ ( CD¹⁹Ă CD²⁰̈́ CD²²) ࠎวّĂҭ CD²³̈́ CD¹⁰( ӈ CALLA ) ࠎౚّĄҭ

˘჌ T ௟ࡪܑᇈ CD⁵ࠎวّ ( ѩᄃ̈୽͐஧௟ࡪ

୽͐ሳТĂҭ׎ C y c l i n D¹ࠎౚّĂ C D^{2 3}ࠎว

ّĂܑ˟^9,20)ĄѩγĂ MCL ͹ࢋ݀ࢨ ( Restriction ) ٺɡ-ᅅᙥĂѣளٺ׎΁ B ௟ࡪ୽͐ሳ۞ k-ᅅᙥ²¹Ą MCL۞ߖҒវૄЯ౵͹ࢋ۞ள૱ߏ t ( 11 Ă 14 )۞ளҜĄт˯ٙࢗĂҭ˵ѣ׎΁۞ត̼Ăт del 13q14 ( 13ߖҒវܜᓖొЊ৿ε )Ăٕ 13q33- 34ă del 8p21 ( 8 ߖҒវൺᓖొЊ৿ε )Ăͽ̈́ del 9p/p15-16 ( 9ߖҒវൺᓖొЊ৿ε )²²Ą

జइડ௟ࡪ୽͐ሳдέ៉۞ಡӘ

ఄඈАಡӘ 21 ּ MCL¹⁹Ăޢ੠ΐࠎ 24 ּ²³Ă

୻˘ҒࠎշّĂ఺ᄃҘ͞۞ 2-4 Ĉ 1 ( M Ĉ F )ѣٙ

̙ТĂ҃έ៉׎΁ܧңڔܛͩ୽͐ሳ۞շּ̃ͧ

ࠎ 1.1 Ĉ 1 Ăιࣇҫٙѣܧңڔܛ͵୽͐ሳ۞ 2%

( ࠹၆ҲٺҘ͞۞ 6%²⁴ )Ă̚มѐ᛬ࠎ 60 ໐Ă࿰

ޢТᇹܧ૱̙рĂ 8 7 % ࠎ୎ഇĄܬϚ۞ጡءͽ

୽͐༼ ( 91% )౵кĂ׎Ѩࠎ੻ល ( 75% )Ăත᝙

ဦˬĈᚢႝّజइડ௟ࡪ୽͐ሳ۞୽͐ඕঽந̷ͯĂ੼

ࢺ(10X40)Ą˯ဦࠎ H & E ߖҒĂ˭ဦࠎҺࠪ௡

ᖐ̼ጯߖҒពϯ CyclinD1 ࠎ८วّĄ

ဦαĈкᇹ̼(Pleomorphic)܂஧តள჌జइડ୽͐ሳ

۞ᆒͩߖҒ(10X100)˯ဦࠎ׹ᙝҕ୵٠ͯĂ˭

ဦࠎ੻ល٠ͯĄ

( 33% )Ăབࡤ྽ ( 25% )̈́ք᝙ ( 16% )Ą׎̚ѣ

˘ּ΍ҋ઼঍ტЪᗁੰĂ֭ѣঽநྋ࣠¹⁹Ą ιࣇѣ۞Ӕனඕ༼ٕᚢႝݭĂ˵ѣ׌ּࠎ܂

஧តளݭĄιࣇ۞ CD5 ŮƝ CD23 ůƝ CD43 Ů Ɲ B C L 2 ŮƝ B C L 6 ůƝ I g D ŮƝ I g M ŮƝ Cyclin D1ŮĂߖҒវѣ 11q²³۞ளҜĂ҃ጱ࡭ B- CL-1ૄЯ۞ࢦ௡Ăซ҃ౄј Cyclin D¹ࠎౚّĂ ҭ׎ Cyclin D²݋ࠎวّ^19,23Ą

జइડ௟ࡪ୽͐ሳ۞ڼᒚ

็௚۞̼ᒚ

M C Lдٙѣ୽͐ᒛ̚ߏ࿰ޢ౵ᗼ۞׎̚˘

჌ĂЯࠎᇾ໤۞̼ᒚߏڼ̙р۞²⁵ ( ࠹၆ٺ׎΁

̚ޘٕ੼ޘೋّ۞௡ཏ )Ąҭ˫̙ညҲޘೋّ۞

˘ཏΞͽᇶ̙ڼᒚ ( Wait & Watch )²⁶ĄЯࠎޢ۰

՟ѣڼᒚ۞̚มх߿ഇࠎ˛ѐĂ҃ MCL Ϊѣ˟

ѐк҃̏Ăٙͽੵܧѐҁវऴ҃࠻੓ֽܜ۞ྵၙ

۞ঽˠ࠰̙ዋϡඈޞᄃ៍၅Ąયᗟߏң჌ڼᒚΞ ͽ଀ז౵р۞ड़ڍĄ

౵૱ϡ۞ߏ CHOP ( Cylophosphamide Ă AdriamycinĂ Oncovin ̈́ Prednisone Ă઼ֹ̰ϡ Prednisolone ²⁷ )Ąιᔵѣ 50% νΠ۞ѣड़தĂҭ

׎൑ঽ̚มх߿ഇ ( Relapse free Survival ) Ϊѣ 7

࣎҃̏͡²⁸Ă̙֭ͧCOP ( ՟ѣAdriamycin ) ࠎָ²⁹Ă னд૱ΐ˯ Rituximab ( R-CHOP )Ąд˘࣎ௐ˟

ഇ۞ྏរᔵѣ 9 6 % ड़தĂҭ൑ঽх߿ഇ˵Ϊѣ 1 6 . 6࣎҃̏͡^{3 0}ĄЯѩѣ׎΁Հк჌ᘽۏĂт cyclophosphamideă doxorubincin ă teniposide ă prednisoneă vincristin ă bleomycin ඈ۞Ъ׀ڼ ᒚ Ą ౵ ܕ ྵ ࠎ ሤ ܝ ۞ ݋ ߏ H y p e r C V A D ( H y p e r f r a c t i o n a t e d c y c l o p h o s p h a m i d eă

vincristină adriamycin ̈́ dexamethasone )Ă֭ᄃ

੼጗ณ methotraxate ̈́ cytarabine Ϲആֹϡ³¹Ą Romagueraඈˠ˫ΐ˯ rituximab Ăඕڍѣ 87%

۞ԆБቤྋ ( C R ) Ă̈́ˬѐ۞൑ঽх߿தࠎ 67%Ăᓁх߿தࠎ 81% Ąҭѣ̣Ҝ ( 5/97 )дڼᒚ

̚Ѫ˸ĂΩѣαҜயϠѨ൴ّϨҕঽٕౄҕ̶̼

̙։া࣏ཏ ( ˬҜ˵Я҃Ѫ˸^31,32 )Ăҭ̏൴ܑ۞

ྤफ़லౣѣࢨĄ

຅௟ࡪொങ

Ϥٺ็௚۞̼ᒚĂ൑ڱѣड़ჯ޺൑ঽх߿

ഇĂٙͽҋវ ( ASCT ) ᄃளវ ( Allogeneic ) ຅௟

ࡪொങಶјࠎΩ˘჌ڼᒚĄ Dreyling ඈ ( ለ߷ M- CL Network³³) д CHOP ٕᙷҬ۞ڼᒚڱޢĂ̶ࠎ ASCTᄃ̒ᕘ৵ ( IFN ) үࠎ׌჌̙Т۞ჯ޺ڼ ᒚĄдΞ̶ژ۞ ( 122/230 )ঽˠ̚Ă൴ᛇ ASCT ᐹٺ IFN Ă݈۰ 81% ྿זቤྋ ( CR )Ăޢ۰Ϊѣ 37%Ą൑ঽх߿ഇ ASCT ࠎ 39 ࣎͡Ă̈́ 54% ˬ ѐх߿தĂ҃ IFN ࠎ 17 ࣎̈́͡ 25% Ą

Gantiඈа໖ّͧྵҋវ ( ASCT ) ᄃளវ ( Allogeneic ) ொങ۞ड़ڍ ( ઼࡚ Nebraska ̂ጯ³⁴ )Ă 80ּତצ ASCT ̈́ 17 ּ Allogeneic ( ࠎΈԙؓ؏

ม ௡ ᖐ ݭ ࠹ ௑ ) Ą ׎ C R , A S C T ࠎ 7 3 % Ă Allogeneicࠎ 62% Ặѐ൑ঽх߿த̶Ҿࠎ 44%

( ҋវ ) ̈́ 49% ( ளវ )Ą̣ѐᓁх߿தࠎ 47%

( ҋវ )Ă࠹၆ٺ 49% ( ளវ )Ă఺ֱӮ൑௚ࢍ˯

۞ࢦࢋमளĄ

Marisඈ˵ဘྏϡ Nonmyeloablative ڼᒚĂ ӈٙᏜ۞ਜ਼ҰொങĂ֭ϡٺ൑ҕቡᙯܼ۞ளវொ

ങĄ൴னޝ͌ѣؼᏵೇ൴۰ĂຍӈΞਕѣொങវ ԩ୽͐ሳ ( Graft-versus-lymphoma ) ۞ड़ڍ³⁵Ą

ࣧؕڼᒚ൑ड़ٕೇ൴ޢ۞ڼᒚ̈́າᘽ۞ण୕

ܑ˟Ĉజइડ௟ࡪ୽͐ሳᄃ׎΁ܧңڔܛͩ୽͐ሳ̝Һܑࠪᇈ۞ͧྵ

ঽ ந ᖎ ᆷ Һܑࠪᇈ

Ćజइડ௟ࡪ୽͐ሳ MCL CD5+, CD23-, CD10Ų, CD43+, CyclinD1+, FMC7+

Ć̈୽͐௟ࡪ୽͐ሳ/CLL SLL/CLL CD5+, CD23+, CD43+, CD11a+, FMC7- Ćል௟ࡪᇹ̈୽͐௟ࡪ୽͐ሳ SLL-pl CD5-, CD23-, CD10-, CD43Ų

Ćᕭڽ͕̚௟ࡪ୽͐ሳ ( all 3 grades ) FCCL CD5-, CD23Ų, CD10+, CD43- Ćᙝቡડ୽͐ሳ ( MALT, MBCL, SMZL ) MZL CD5-, CD23-, CD10-, CD43Ų

Ćත̝ඖͨې୽͐௟ࡪ୽͐ሳ SLVL CD5-, CD11cŲ, FMC7+, CD22+, CD24+

Ćкͨ௟ࡪϨҕঽ HCL CD5-, CD10-, CD25+, CD11c+, CD103+, B-ly-7+

ොĈ MALT, ᕆቯ௡ᖐ࠹ᙯ̝୽͐ሳć MBCL, ᙷಏ८ B ௟ࡪ୽͐ሳć SMZL, ත̝ᙝቡડ୽͐ሳĄ

дೇ൴ٕࣧؕڼᒚ൑ड़۞ଐڶ˭Ă Forstpointer അϡ F l u d a r a b i n ă C y c l o p h o s p h a m i d e ̈́ Mitoxantroneᄃ Rituximab ( R-FCM )³⁶Ă൴ன R-FCMᐹٺ FCM Ą̚มх߿ഇࠎ 11 ࣎͡( FCM )Ă

҃ R-FCM ݋إϏ྿זĄГֽಶߏ Rituximab ă E t o p o s i d eă P r e d n i s o n e ă Vi n c r i s t i n e ă Cyclophosphamideă Doxorubicin ( R-EPOCH )³⁷Ă

̚มх߿ഇࠎ 15 ࣎͡Ă˵ѣ׎΁۞Ъ׀̼ᒚĄ غ˭ࢋ̬௜າᘽ B o r t e z o m i b^{3 8 , 3 9}Ăιߏ Proteosome۞Ժט጗Ă׎Ժטጱ࡭к჌௟ࡪ̶ෘ

አ༼కϨ ( Β߁ Cyclins ) ۞ુ᎕ĄΩ˘࣎Ξਕ፟

ᖼࠎۡତ͔൴௟ࡪѪ˸ ( Apoptosis )Ăѩ˜གྷϤ ԩ ( bax ) ̈́ܳ ( bix ) አଠѪ˸కϨ۞፟ᖼĄΩ˘

࣎ͧྵ୻຾۞፟ᖼߏԺט NF-ɠ B Ăιߏ˘჌ᖼ ᛌЯ̄Ą҃ Proteosome ਕܡͤι۞ҋ൒Ժט጗

( Iɠ B ) ۞৔ᗼĂซ҃Ժט NF-ɠ B ۞߿̼Ąᓜ Ԗ˯ᓁѣड़த ( O R R ) ࠎ 4 4 % Ă׎̚ C R ࠎ 18%Ą઼࡚ FDA ̏८ࣞ׎ϡдк൴ّ੻លሳ̈́

MCL۞ڼᒚĄ

ඕኢ̈́Ϗֽण୕

M C Lдܧңڔܛͩ୽͐ሳ̚ߏޝপள۞ֲ

ཏĂঽந࠻ֽ֭ܧޝೋّĂҭܜ଀ݒͧᕭڽݭ୽

͐ሳࠎԣĂ҃ڼᒚ۞ѣड़ഇݒᅈ̙т̚ޘٕ੼ޘ

ೋّ۞˘ཏĂјࠎ౵ᙱڼᒚ۞୽͐ሳĄι۞ᓜԖ

࿰ޢͽ MIPI ҿᕝྵ IPI ̈́ FLIPI ໤ቁĄፂඊ۰ռ

˭ᄃ઼࡚ Sloan-Kettering ( ৸ࡗ ) ̈́ MD Anderson ( ᇇэ ) ׌̂ᒛা͕̚۞ତᛈĂ΁ࣇౌޙᛉтڍ ঽˠྵѐᅅͷ׎΁ᗁڶధΞ۞ྖ, дௐ˘ѨԆБ ቤྋ̝ޢઇ຅௟ࡪொങĄ

ཚሳᗁጯ൑ႷгдซՎ̝̚Ă௢᎕࿅Ν۞ј Αâᕇ˘ႍ۞Ш݈ซणĄன̫Һࠪڼᒚă̼ᒚă

຅௟ࡪொങౌдซՎĄᔘѣາᘽ۞൴णĂ၆୽͐ሳ јঽ۞ᄮᙊͽ̶̈́̄ᇾ࿯۞ڼᒚĂԯͽ˯఺ֱາۢ

Ъ׀ፋЪĂԓ୕ਕѣٙࡎ৔Ą֭୕ѣԠ˘Т۞ᗁर ਕઇྭੰᅫ۞ᓜԖྏរͽԱ΍౵р۞ڼᒚĄ

࡭ᔁ

ຏᔁเኰਕᗁर೩ֻঽந໰ͯĄ

ඊ۰ᓏځώ͛ϏצЇңᇄથ̝ᙒӄĂ֭൑Ї ң̝Ӏৈ኏ࡎ

ણ҂͛ᚥ

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Mantle Cell Lymphoma, It Appears Indolent but Actually Aggressive, also Summary the

Classification of Non-Hodgkin's Lymphoma

Chung-King Lin^1,3, and Shih-Hung Huang²

Mantle cell lymphoma (MCL) is recognized "with evidence" as a subtype of non-Hodgkin's lymphoma in early 1990s. It was categorized mostly in the group of diffuse small lymphocytic / small cleaved cell lymphomas before this recognition. Therefore, it might not seem so aggressive morphologically. But in fact, it behaved very differently from other diseases with similar morphology. The median survival without treatment is much shorter than other kinds of low grade lymphoma. When it is treated conventionally, the failure free survival is much short- er as compaired to aggressive lymphomas (e.g. diffuse large B cell lymphoma) and there is no cure at all. Genetic and immunophenotypic features are characterized by overexpression of Cyclin D1 secondary to translocation of t(11;14)(q13:q32). The pan B-antigens, CD19, CD20, CD22 are positive but CD23 is negative. CD10 (CALLA) is usually negative and coexpression of pan T antigen CD5 and its median age of occurrence is 58. It has male pre- dominance clinically. The common signs of presentation are generalized lymphadenopathies(71%-90%), hep- atosplenomegaly and bone marrow involvement(53%-90%). Involvement of G-I tract is strikingly high in nearly all cases. The prognostic evaluation by multivariate analysis showed the new mantle cell lymphoma internation- al prognostic index (MIPI) was related to survival and worked better. MCL is incurable with conventional chemother- apy. So stem cell transplant should be considered on first remission on a medical fit patient. From retrospective reviews, there was no statistically significant difference in 5 year survival rates compared the autologous(47%) to allogeneic(49%) transplants. ( J Intern Med Taiwan 2008; 19:491-498 )

1Division of Hematology-Oncology,

2Division of Pathology, Cathay General Hospital, Taipei, Taiwan;

3Taipei Medical University, Taipei, Taiwan