Ъ‫׀‬ϩቲ‫ܬ‬Ϛ̝ࣧ൴ّБّ֗ ੜҖ൴ֈ௟ࡪ୽͐ሳ ę ঽּಡӘ ę ‫إڒ‬Ԡ

߸ҹ‫ځ‬

ૺ‫ځ‬Ԡ*

έΔξϲ‫م‬Ԃᗁੰ ੺ઌࡔ‫ه‬ᗁੰ

໢৵ሪ

ϩቲࡊ

ҕ୵ࡊ*

Primary Systemic Anaplastic Large Cell Lymphoma with Skin Involvement — A Case Report — Shang-Chih Lin

Ke-Ming Hung

Ming-Chih Chang*

Su-Ying Wen

Anaplastic large cell lymphoma (ALCL) is a rare non-Hodgkin’s lymphoma. Primary systemic anaplastic lymphoma kinase - negative ( ALK - ) ALCL has a poor response to chemotherapy and an unfavorable prognosis in contrast with primary systemic ALK + ALCL. We report a 93-year-old man presented with three dusky erythematous papules on left supraclavicular area and a rapidly enlarging erythematous tumor on left chest wall for about half a month. The histopathological examination showed a patchy and nodular infiltrate of a mixed population of lymphoid cells ranging from reactive small lymphoid cells to large anaplastic cells in the dermis and subcutis. The immunohistochemical study revealed positivity of neoplastic cells for CD30 antigen and negativities for CD3ăCD4ă CD5ăCD15ăCD20ăCD56ăALK antigens. Abdominal CT scan and gallium-67 whole body scan showed involvement of right claviclar lymph nodes, mediastinum and spleen. Based on the clinicopathological and immunohistochemical findings, a diagnosis of primary systemic anaplastic lymphoma kinase-negative ALCL with skin involvement was made. He was treated with polychemotherapy and partial remission was achieved. We hereby report this case and review the literature. (Dermatol Sinica 21 : 367-374, 2003) Key words: Anaplastic large cell lymphoma, Anaplastic lymphoma kinase, CD30

From the Department of Dermatology, Chung-Hsiao Municipal Hospital and the Department of Hematology*, Mackay Memorial Hospital Accepted for publication: April 02, 2003 Reprint requests: Shang-Chih Lin, M.D., Department of Dermatology, Chung-Hsiao Municipal Hospita, 87, Tong-Teh Road, Nan Kang, Taipei, Taiwan, R.O.C. TEL: 886-2-27861288 FAX: 886-2-27888492

367

‫إڒ‬Ԡ

ੜҖ൴ֈ̂௟ࡪ୽͐ሳߏ˘჌͌֍۞়ঽć࠹ྵ‫ܑٺ‬ன΍ ALK ᏉЪకϨኳ۞ࣧ൴ّБ֗ ّ ALCL ঽּĂࣧ൴ّБّ֗ ALCL ܑ̙ன΍ ALK ᏉЪకϨኳ۞ঽּ၆‫̼ٺ‬ጯ‫ڼ‬ᒚѣྵम۞ ͅᑕͽ̈́ྵम۞࿰‫ޢ‬Ă ӍˠಡӘ˘Ҝ 93 ໐շّଈ۰Ă̂ࡗΗ݈࣎͡Ăдν઎ᗆ੻˯ొҜ൴ னˬ࣎ຳࡓҒ̈͸ৃͽ̈́дν઎਒ጨϩቲ˘࣎֝ి‫۞̂ܜ‬ཚ๴Ą௡ᖐঽநጯᑭߤពϯৌϩᆸ ᇃ‫ھ‬гওማ඾ᇴ჌୽͐௟ࡪĂ఺ֱ୽͐௟ࡪΒ߁̈୽͐஧ͽ̈́̂҃ੜҖ൴ֈ۞ཚሳ௟ࡪĄҺ ࠪ௡ᖐ̼ጯᑭߤពϯ఺ֱཚሳ௟ࡪ၆ CD30 ߖҒӔวّĂ҃၆ CD3ăCD4ăCD5ăCD15ă CD20ăCD56ăALK ߖҒӔౚّĄཛొ࿪ཝᕝᆸବႿ̈́㈤ 67 ८̄ᗁጯᑭߤពϯΠ઎ᗆ੻ొҜ ୽͐ඕă਒ొᓂჽට̈́ත᝙ѣ୽͐ሳ‫ܬ‬ϚĄԧࣇ෧ᕝ఺ߏ˘ּЪ‫׀‬ϩቲ‫ܬ‬Ϛ̝ࣧ൴ّБّ֗ ੜҖ൴ֈ୽͐ሳ፬∰ౚّੜҖ൴ֈ̂௟ࡪ୽͐ሳĄঽଈତ‫צ‬кࢦ̼ጯ‫ڼ‬ᒚĂ‫ڼ‬ᒚҌ̫ 7 ࣎ ͡Ăϫ݈఍‫̶ొٺ‬ቤྋ‫ې‬ၗĂཚሳঐε 75 % ֭ᚶᜈ‫ܝ‬෧੠ᖸ‫ڼ‬ᒚĄ( ̚රϩᄫ 21 : 367 - 374, 2003 )

݈

֏

ཏٙၹјĂ‫ݒ‬ѣ඾̙Т۞ᓜԖܑனͽ̈́Һ

ੜҖ൴ֈ̂௟ࡪ୽͐ሳ ( anaplastic

ࠪጯܑ‫ݭ‬ĂࠤҌၟ൒̙Т۞࿰‫ ޢ‬2, 3Ąдѩ

large cell lymphomaĂᖎჍ ALCL ) ֶፂ The

ԧࣇಡӘ˘ּЪ‫׀‬ϩቲ‫ܬ‬Ϛ̝ࣧ൴ّБ֗

Revised European - American Classification of

ّੜҖ൴ֈ̂௟ࡪ୽͐ሳĂдѩ̝݈Ăϩ

Lymphoid Neoplasm ( REAL ) ̶ᙷ Ăߏ˘჌

ᄫϏഅѣ࠹ТঽּಡӘĂТॡώ͛૟аᜪ

Ϥ T ௟ࡪٕ‫ ܧ‬B ‫ ܧ‬T ௟ࡪ ( null cell ) ̶̼҃

͛ᚥĄ

1

ֽ۞‫ܧ‬ң‫͐୽ͩܛڔ‬ሳĂ‫׎‬ཚሳ௟ࡪܑன ΍CD30 ԩࣧĄALCL ߏ˘჌͌֍۞়ঽĂ ࿅ΝALCLజᄮࠎߏϤ‫׍‬ѣ࠹Ҭপᇈ۞়ঽ

ঽּಡӘ ˘Ҝ 93 ໐շّĂ‫ٺ‬ϔ઼ 91 ѐ 3 ͡ 4 ͟

Fig. 1

Fig. 2

A rapidly enlarging erythematous dome-shaped tumor with about 2.5X3 cm in size on left chest wall for about half a month.

Three erythematous bean-to-pea-sized papules on left supraclavicular area for about half a month.

368

Dermatol Sinica, December 2003

Ъ‫׀‬ϩቲ‫ܬ‬Ϛ̝ࣧ൴ّБّ֗ੜҖ൴ֈ‫̂ې‬௟ࡪ୽͐ሳ

Fig. 3

Fig. 4

Skin biopsy of the papule on supraclavicular area showed a patchy and nodular infiltrate in the dermis and subcutis.(H & E, x10)

The infiltrate is composed of a mixed population of lymphoid cells ranging from reactive small lymphoid cells to large anaplastic cells. (H & E, x200)

ҌώࡊՐ෧Ă͹෦̂ࡗ˘ฉ݈൴னν઎਒

ϩቲ̷ͯᑭߤĄ࿅‫ـ‬ঽΫ͞ࢬĂঽˠ‫ ٺ‬5 ѐ

ጨϩቲѣ˘࣎ຳࡓҒສᚧ۞๪౤‫ې‬೹๴Ă

݈̚ࢲĂ̝‫ޢ‬Ӕν઎ઐឨĂੵѩ̝γ֭൑

ϩቲᑭߤ൴னĈν઎਒ጨຳࡓҒ۞೹๴̂

‫΁׎‬ঽΫĄ

̈̂ࡗ 1.5 ̶̳ X 2.5 ̶̳Ăܑࢬѣᒴᜀត

ϩቲঽந̷ͯពϯĈܑϩᆸ֭Ϗ‫צ‬ཚ

̼ĂТॡν઎ᗆ੻˯ొҜĂ൴ன ˘࣎ 3 ̳

ሳ௟ࡪ‫ܬ‬ϚĂৌϩᆸᇃ‫ھ‬гওማ඾ᇴ჌୽

Ე̂̈۞ຳࡓҒ͸ৃĂᛈ෧ॡ൴னѩ͸ৃ

͐௟ࡪ Ă఺ֱ௟ࡪওማଂৌϩᆸ‫ؼ‬ҩҌϩ

˭͞ѣ˘࣎ဩ̙ࠧ‫۞ځ‬ϩ˭ওማ೹๴Ăᓜ

˭௡ᖐ ( Fig. 3 )Ă఺ֱ୽͐௟ࡪΒ߁̈୽͐

Ԗ˯ѣϩቲ‫ۆ‬Ъ‫ܑ׀‬ϩᒴᜀன෪҃Аග̟

஧Ă̂҃ੜҖ൴ֈ۞ཚሳ௟ࡪĂٕᙷҬ

૵ৼΞ֯ჼ ( neomycin, triamcinolone ace-

Reed - Sternberg ௟ࡪ ( ᖎჍ RS ௟ࡪ ) ۞λ̂

tonide, mycostatin ) Ԋొ๮٠֭ឤ‫׳‬ঽଈа ෧ćঽଈ‫ ٺ‬91 ѐ 3 ͡ 11 ͟а෧ॡᑭෛঽի ܑϩᒴᜀ̏ԼචĂҭν઎਒ጨຳࡓҒ۞೹ ๴֝ి‫֭̂ܜ‬ษ੓Ӕ๪౤‫ې‬ཚ๴̂̈̂ࡗ 2.5 X 3 ̶̳ ( Fig. 1 )ĂТॡν઎ᗆ੻˯ొ ҜĂੵ˞ࣧА۞͸ৃൾត̂Ҍۡशࡗ 8 ̳ ᲔĂΩ൴ன ‫࣎׌‬າ‫ܜ‬΍۞ຳࡓҒ͸ৃ ( Fig. 2 )Ă఺ֱ͸ৃ˭͞۞ϩ˭ওማ೹๴˵ត̂ Ҍ̂ࡗ 3 X 4 ̶̳̂̈Ąநጯᑭߤ൴னν઎ ᐚొѣˬ࣎ࡗ 1 ̶̳̂Ăᛈ෧ॡΞொજ̙൭ ۞ϩ˭ඕ༼Ă֭൑քතཚ̂ன෪ĄᓜԖ˯ ᘃႷߏ୽͐ሳĂٕ‫΁׎‬ᖼொّཚሳĄ̶Ҿ

Fig. 5

‫ٺ‬ν઎ᗆ੻˯۞͸ৃ‫׶‬ν઎਒ጨ۞ཚ๴ү

Tumor cells demonstrated positivity for CD30. (CD30, x200)

Dermatol Sinica, December 2003

369

‫إڒ‬Ԡ

Fig. 6

Fig. 7

Abdominal CT scan showed slight enlargement of the spleen with multiple lowly enhanced area scattered throughout the spleen.

Gallium-67 whole body scan showed abnormal tracer uptake in the right pulmonary hila, right supraclavicular area and lateral infraclavicular area.

௟ࡪĄ ఺ֱ̂҃ੜҖ൴ֈ௟ࡪѣ୻຾۞८

఍‫̶ొٺ‬ቤྋ‫ې‬ၗĂཚሳঐε 75 % ֭ᚶᜈ

̥Ăͽ̈́౅‫۞ܪ‬௟ࡪኳ ( Fig. 4 )ĄপঅߖҒ

‫ܝ‬෧੠ᖸ‫ڼ‬ᒚĄ

Ӕன CD30 ԩࣧวّ ( Fig. 5 )Ą‫ ΁׎‬CD3ă CD4ăCD5ăCD15ăCD20ăCD56ă

੅

ኢ

anaplastic lymphoma kinase ( ᖎჍ ALK ) ԩࣧ

1982 ѐ Schwab ඈˠ 4 дң‫͐୽ͩܛڔ‬

‫݋‬ӔౚّͅᑕĄ၁រ‫ވ‬ᑭߤ͞ࢬĂঽˠ۞

ሳ ( Hodgkin's lymphoma ) ۞ RS ௟ࡪ˯൴ன

3

WBC: 8720 / mm ( Neutrophil: 78.4 %ƓĂ

፾প۞າ̶̄Ă֭૟‫׻׎‬Щࠎ Ki -1 ԩࣧ

Lymphocyte: 12.7 %ƔĂEosinophil: 0.7 % )Ă

( ᐌ‫ޢ‬జ‫׻‬ЩࠎCD30 )Ąд 1985 ѐ Stein ඈ

Hgb: 10.5g / dlĂMCV: 87.7fLĄཛొ࿪ཝᕝ

ˠ5 Հซ˘Վ൴னĂд 45 ҜੜҖ൴ֈ۞̂௟

ᆸବႿ̈́㈤ 67 ८̄ᗁጯᑭߤពϯΠ઎ᗆ੻

ࡪ୽͐ሳঽּ‫׎‬ཚሳ௟ࡪӮӔன Ki -1 ԩࣧ

ొҜ୽͐ඕă਒ొᓂჽට̈́ත᝙ѣ୽͐ሳ

วّͅᑕćд௡ᖐঽந̷ͯ˭Ă఺ֱཚሳ

‫ܬ‬Ϛ ( Fig. 6, 7 )Ą

௟ࡪӮӔனੜҖ൴ֈ۞γ៍ă้Ш‫ٺ‬጖ჸ

ტЪᓜԖܑன̈́ঽந௡ᖐត̼Ăԧࣇ

гϠ‫ܜ‬ă̈́‫ܬ‬Ϛ୽͐ඕᚭĄ΁ࣇ૟‫׍‬ѣ˯

෧ᕝࠎЪ‫׀‬ϩቲ‫ܬ‬Ϛ̝ࣧ൴ّБّ֗ ALK

ࢗপᇈ۞୽͐ሳ‫׻‬ЩࠎੜҖ൴ֈ̂௟ࡪ୽

-

ALCLĂĄώঽּПᐍ࿰‫޽ޢ‬ᇴᛳ‫੼ٺ‬Пᐍ

͐ሳ ( ALCL ) 4Ą

ཏͷវਕ‫ې‬ၗ̙р߇൑‫ڱ‬ઇ᎕ໂّ̼ጯ‫ڼ‬

ጐგ ALCL Я‫׍‬ѣ˯ࢗপᇈ҃జ̶ᙷ

ᒚĂགྷග̟̼ጯ‫ڼ‬ᒚ cyclophosphamide

ࠎ˘፾ϲ۞়ঽ ( entity )ĂALCL ۞ঽּд

800mgă epirubicin 50mgă vinblastin

ᓜԖ˯ă௟ࡪԛၗጯ˯ăҺܑࠪ‫˯ݭ‬Ӕன

1.5mgăhigh dose prednisolone 4 Ѩ‫ޢ‬Լϡ

ளኳّ 2Ąౙᜈ۞ࡁտᄃಡӘ൴னĈ˘ొ̶

etoposide ̈́ cyclophosphamide ΐ˯ pred-

۞ ALCL ঽּᄃߖҒវ۞Ϲ̢ொҜѣᙯĂ

nisolone ˾‫ڼڇ‬ᒚĂ‫ڼ‬ᒚҌ̫ 7 ࣎͡Ăϫ݈

఺ֱϹ̢ொҜ͹ࢋߏ t ( 2;5 ) ( p23;q35 ) 6Ą఺

370

Dermatol Sinica, December 2003

Ъ‫׀‬ϩቲ‫ܬ‬Ϛ̝ࣧ൴ّБّ֗ੜҖ൴ֈ‫̂ې‬௟ࡪ୽͐ሳ

Table I. Primary ALCL of T or null phenotype: correlation between morphology, ALK expression and clinical features 9.

Type Systemic ALCL ALK-Positive (~60%) Systemic ALCL ALK-negative (~40%) Primary cutaneous ALCL

Morphology All variants (common type to small cell) Mostly common type and giant cell Common type to lymphomatoid papulosis

ALK protein Present

Age First three Decades

Absent

Usually old Patients

Absent

Usually old patients

Clinical Features Frequently stage III-IVDisease with systemic Symptoms Frequently stage III-IV Disease Isolated or multiple skin Nodules. No systemic symptoms

Outcome Usually good

Poor

Usually good

ֱߖҒវϹ̢ொҜٙౄјᏉЪૄЯᖼᛌ҃

ࣧ൴ّБّ֗ ALK + ALCL дᓜԖ

ј۞ᏉЪకϨኳӣѣੜҖ൴ֈ୽͐ሳ፬∰

ܑனăҺࠪጯܑ‫ݭ‬ă௡ᖐԛၗܑᇈ

( anaplastic lymphoma kinaseĂᖎჍ ALK ) ۞

࠹ҬĂΪਕͽ ALK కϨኳߏӎܑ

ొ̶ͯ߱ ĄՀซ˘Վ۞ಡӘពϯĂд

னડҾĄѩ‫఼ݭ‬૱൴Ϡ‫ྵٺ‬ѐ‫۞ܜ‬

ALCL ܑன΍ ALK ᏉЪకϨኳ۞ঽּĂѣ

ঽˠĂ൑‫ځ‬ពّҾमளĂѣಡӘ‫׎‬

࠹Ҭྵָ۞࿰‫ޢ‬Ăͽ̈́၆̼ጯ‫ڼ‬ᒚѣྵָ

྿ௐ 3 ഇăௐ 4 ഇ۞ͧதྵҲ 10ćѩ

۞ͅᑕ 3 ĄΩѣࡁտಡӘពϯࣧ൴ّϩቲ

γĂ࿰‫ྵޢ‬म 10Ą

7

ALCL ˘ਠѣྵָ۞࿰‫ޢ‬ĂͷӮܑ̙ன΍

c ࣧ൴ّϩቲ ALCLĈ఼૱൴Ϡ‫ٺ‬ҁ

ALK కϨኳĂΞజෛࠎ˘፾ϲ۞়ঽ Ąፂ

ѐˠĂπӮ 60 ໐ĂALK ఼૱Ӕன

ѩĂֶፂᓜԖܑᇈ̶̈́̄ጯᇾ໤Ăࣧ൴ّ

ౚ ّ 8ć ᓜ Ԗ ˯ Ă ͽ ಏ ˘ ٕ к ൴

ALCL ΞͽГડ̶ࠎ 3 ࣎፾ϲ۞়ঽĂѩ̶

ّĂ൑া‫ࢬܑې‬ट‫ٽ‬ሚႹ۞඘ࡓҒ

ᙷు႙ࠎᇃ‫ھ‬ତ‫ ( צ‬Table I )Ĉ

ϩቲཚሳܑனĂ̂ࡗ 25 % ঽּĂົ

8

9

a ࣧ൴ّБّ֗ALK ALCLĈ̂к൴

ԆБٕొ̶ҋҖቤྋĂ૟ঽիԆБ

Ϡ‫ ٺ‬20 Ҍ 39 ໐۞ѐᅅշّĂ൴ன

̷ੵ‫ޢ‬ᏴፄЪ‫̙ٕ׀‬Ъ‫ٸ׀‬ड‫ڼ‬

ॡ̂к̎྿ௐ 3 ٕௐ 4 ഇĂ֭གྷ૱

ᒚĂΞ଀‫ז‬ໂָ۞࿰‫ ޢ‬8Ą

+

Ъ‫׀‬Б֗া‫ ې‬10Ą̂ࡗ 60 % ۞ঽּ

ϩቲࡊ૱֍۞ ALCL ͽࣧ൴ّϩቲ

ѣ୽͐ඕͽγ۞‫ܬ‬ϚĂ‫ͽ̚׎‬ϩቲ

ALCL ঽּྵкĂঽի݀ࢨ‫ٺ‬ϩቲ҃՟ѣ

౵૱‫ܬצ‬Ϛ ( 21 % )Ă‫׎‬Ѩߏ੻ᐝ

Бّ֗‫ܬ‬Ϛćώঽּੵ˞ϩቲѣ֝ి‫̂ܜ‬

( 17 % )Ăహ௡ᖐ ( 17 % ) Ă၆‫̼ٺ‬

۞ཚ๴̈́͸ৃĂᗆ੻ొҜ୽͐ඕ̈́਒ొᓂ

ጯ‫ڼ‬ᒚѣྵָ۞ͅᑕ̈́࿰‫ ޢ‬Ą

ჽට̈́ත᝙˵ѣ୽͐ሳ‫ܬ‬ϚĂALK Ӕனౚ

10

3

b ࣧ൴ّБّ֗ ALK ALCLĈѩ‫ݭ‬ᄃ -

Dermatol Sinica, December 2003

ّĂᑕᛳ‫ࣧٺ‬൴ّБّ֗ ALK - ALCLĄ

371

‫إڒ‬Ԡ

д௡ᖐঽநጯ˯ĂALCL Ξ̶ࠎĈ૱

type A Ξ֍‫͌ז‬ณ̂۞ளԛ௟ࡪ 14ć ALCL

֍‫ ( ݭ‬common / classic type )ă̈௟ࡪ‫ݭ‬

д௡ᖐঽநጯ˯‫݋‬Ӕனᇴณྵк̂۞ள‫ݭ‬

( small cell type )ă୽͐௡ᖐ஧‫ ( ݭ‬lympho-

௟ࡪĂགྷ૱Ӕனͯ‫ې‬ওማĂ֭ͷྵ૱ѣ۞

histiocytic type )ăλ̂௟ࡪ‫ ( ݭ‬giant cell

к८௟ࡪ 11Ă̂۞ள‫ݭ‬௟ࡪ۞ᇴณߏડҾᙯ

type )Ą૱֍‫ ݭ‬ALCL1, 5, 11 д H & E ߖҒ˭Ă

ᔣĄ

Ξ֍‫҃̂ז‬кԛّ‫׍‬ѣᖳಱ௟ࡪኳ۞ཚሳ

ң‫͐୽ͩܛڔ‬ሳдϩቲ۞ঽի̂кߏ

௟ࡪĂӔனͯ‫ ( ې‬sheet ) Ϡ‫ܜ‬Ăιࣇ۞௟ࡪ

Ϥ୽͐ඕ‫ؼ‬ҩ‫ܬ‬ϚϩቲĂٕߏҜ‫ٺ‬۳ბ‫ܕ‬

८གྷ૱ߏ੺Ꮶ‫ݭ‬ͷ‫׍‬ѣк࣎̈҃๝ែّ۞

઎۞୽͐ඕа໖ّ‫ܬ‬Ϛྍ۳ბ۞ϩቲঽի15

८̥Ă‫׍‬ѣ˯ࢗ௟ࡪጯԛၗ۞௟ࡪΞ֍‫ٺ‬

Ăώঽּϩቲ۞ঽի̶ཏ̶ο‫ٺ‬ν઎ᗆ੻

Ч‫ ݭ‬ALCLĂ߇జჍࠎߏপᇈ௟ࡪ ( hall-

˯ొҜͽ̈́ν઎਒ጨϩቲĄд௡ᖐঽந

mark cell ) ćѣॡΞͽ֍‫ז‬к८ٕӔன RS

ጯĂң‫͐୽ͩܛڔ‬ሳ˯ͽ RS ௟ࡪٕ‫ܛڔݣ‬

‫ې‬௟ࡪ΍னĄ఺ֱཚሳ௟ࡪ఼૱஄Ъ൴‫ۆ‬

ͩ௟ࡪࠎপᇈćRS ௟ࡪĂӔனᗕ८Ă̂҃

௟ࡪওማĂтĈ௡ᖐ஧ăል௟ࡪă๝Ͽࡓ

۩‫۞ېڽ‬௟ࡪ८Ăჸะјဥ۞௟ࡪኳ̙̈́

ّ஧ăкԛ८Ϩҕ஧ඈĄώঽּ۞௡ᖐঽ

ఢ‫۞݋‬८ቯă८̥Ӕன๝Ͽࡓّ҃ͷ౅‫ܪ‬

ந̷ͯΞ֍‫ז‬Ĉдৌϩᆸᇃ‫ھ‬гওማ඾ᇴ

֭̂̈෹࿅௟ࡪ८ۡश۞ 1 / 3ćң‫ͩܛڔ‬௟

჌୽͐௟ࡪĂ఺ֱ୽͐௟ࡪΒ߁̈୽͐

ࡪ‫ߏ݋‬ಏ८Ăҭ̪‫׍‬ѣ RS ௟ࡪপᇈ۰ 15Ą

஧Ă̂҃ੜҖ൴ֈ۞ཚሳ௟ࡪĂٕᙷҬ RS

࠹ྵ‫ ٺ‬ALCL ‫۞̂ͽ݋‬ੜҖ൴ֈ௟ࡪࠎপ

௟ࡪ۞λ̂௟ࡪćপঅߖҒӔன CD30 ԩࣧ

ᇈĂѣॡΞ֍‫͌ז‬ᇴ Reed - Sternberg ‫ې‬௟

วّĂ၆ CD3ăCD4ăCD5ăCD15ă

ࡪĄѩγĂRS ௟ࡪ̈́ң‫ͩܛڔ‬௟ࡪ˘ਠӔ

CD20ăCD56 ԩࣧߖҒ‫݋‬ӔౚّͅᑕĂព

ன CD15 ԩࣧวّͅᑕĂ࠹ͅгĂALCL ‫݋‬

ϯཚሳ௟ࡪ۞ԩࣧѣ௲εன෪ć௟ࡪওማ

Ӕன CD15 ԩࣧౚّͅᑕ 14Ăт‫ڍ‬௟ࡪԛၗ

͹ࢋߏͽ̂҃ੜҖ൴ֈ۞ཚሳ௟ࡪЪ‫׀‬஄

̪൑‫ڱ‬ડҾĂCD15 ԩࣧপঅߖҒΞͽᅃӄ

Ъ൴‫ۆ‬௟ࡪၹјĂͷώঽּᙷҬ RS ௟ࡪ۞

ᝥҾ෧ᕝĄ

12

λ̂௟ࡪᇴณໂ͌Ă֭ͷд௟ࡪጯԛၗ

ѩγĂҺࠪ‫܂‬௟ࡪ୽͐ሳ ( immunob -

˯Ă఺ֱ̂҃ੜҖ൴ֈ۞ཚሳ௟ࡪ۞ᄃপ

lastic lymphoma ) ᄃโҒ৵௟ࡪሳд௟ࡪԛ

ᇈ௟ࡪ࠹௑ĂტЪ˯ࢗপᇈӮᄃ૱֍‫ݭ‬

ၗ˯ᄃ ALCL ࠹ҬĂҭ‫׌‬۰ӮӔன CD30 ԩ

ALCL ࠹௑Ą

ࣧౚّͅᑕĄ

дᝥҾ෧ᕝ͞ࢬĂALCL ͹ࢋυืᄃ

д‫ڼ‬ᒚ̈́࿰‫ࢬ͞ޢ‬Ăࣧ൴ّБّ֗۞

୽͐ሳᇹ͸ৃা ( lymphomatoid

ALCL ଳϡ cyclosohosphamide, doxorubicin,

papulosis )ăң‫͐୽ͩܛڔ‬ሳăҺࠪϓ௟ࡪ

vincristine, prednisolone ( CHOP ) ̼ጯ‫ڼ‬ᒚ

୽͐ሳ ( immunoblastic lymphoma )ăโҒ৵

̂ࡗ 48 % ঽּΞ‫ܐ‬Ѩቤྋ 16ć‫ ׎‬5 ѐх߿த

௟ࡪሳүᝥҾ෧ᕝĄ

д ALK + ̈́ AlK- ̶Ҿߏ 71 % ̈́ 15 % 10ĄΩѣ

୽͐ሳᇹ͸ৃাᓜԖ˯̂кͽк࣎୶

ಡӘଳϡ 5 - fluorouracil, methotrexate, cyto-

ࡓҌഊҒ۞͸ৃܑனĂঽ඀ྵ̙߿᜙ ( indo-

sine arabinoside, cyclosohosphamide, doxoru-

lent ) Ăᄃώঽּ֝ిซण̙ТĄд௡ᖐঽ

bicin, vincristine, prednisolone ( F - MACHOP )

நጯ˯Ă୽͐ሳᇹ͸ৃাડ̶ 2 ‫ݭ‬Ă‫̚׎‬

̼ጯ‫ڼ‬ᒚ‫ޢ‬т‫ڍ‬ѣണ঻Ԋొঽի‫݋‬ᅃͽ‫ٸ‬

13

372

Dermatol Sinica, December 2003

Ъ‫׀‬ϩቲ‫ܬ‬Ϛ̝ࣧ൴ّБّ֗ੜҖ൴ֈ‫̂ې‬௟ࡪ୽͐ሳ

ड‫ڼ‬ᒚĂ౵‫ޢ‬ٙѣঽଈӮତ‫צ‬ҋវ੻លொ തĂ఺჌ాᜈ૜ะ۞‫ڼ‬ᒚ ( sequential intensive therapy ) ΞᔖҺѝഇೇ൴֭‫ܜؼ‬х߿ഇ ม 17Ąώঽּଈ۰ѐࡔ੼ă̶ഇ੼ăПᐍ࿰ ‫޽ޢ‬ᇴᛳ‫੼ٺ‬Пᐍཏăវਕ‫ې‬ၗ̙рͽ̈́ ᛳ‫ࣧٺ‬൴ّБّ֗ ALK - ALCL ߏ၆‫̼ٺ‬ጯ ‫ڼ‬ᒚѣྵम۞ͅᑕ̈́࿰‫ޢ‬Ą аᜪώঽּಡӘĂ֝ి‫۞̂ܜ‬ϩቲཚ ๴ᑕྎ௟ય෧ăநጯᑭߤٕซ˘Վঽந̷ ͯᑭߤĂͽଵੵ୽͐ሳٕ‫΁׎‬ᖼொّཚ ሳĄѩγĂͽϩቲ‫ܬ‬Ϛܑன̝ࣧ൴ّБ֗ ّ ALK - ALCL ၆̼ጯ‫ڼ‬ᒚѣྵम۞ͅᑕͷ ࿰‫ྵޢ‬मĂ‫ ׎‬5 ѐх߿த̂ࡗΪѣ 15 % 10ć дࣧ൴ّϩቲ ALCLĂ̂ࡗ 25 % ঽּົԆ Бٕొ̶ҋҖቤྋĂ૟ঽիԆБ̷ੵ‫ޢ‬Ᏼ ፄЪ‫̙ٕ׀‬Ъ‫ٸ׀‬ड‫ڼ‬ᒚĂΞ଀‫ז‬ໂָ۞ ࿰‫ ޢ‬8 ĄᓜԖ˯Тᇹͽϩቲ͸ৃٕཚ๴ܑ னĂࣧ൴ّБّ֗ ALK - ALCL ᄃࣧ൴ّϩ ቲ ALCL ‫ڼ׎‬ᒚ͞ёă‫ڼ‬ᒚͅᑕ̈́࿰‫˵ޢ‬ ѣ࠹༊̂۞मளĂ‫ߏ̚׎‬ӎ݀ࢨ‫ٺ‬ϩቲٕ ѣБّ֗‫ܬ‬ϚăALK ܑனᄃӎߏϫ݈̏ۢ ཚሳෞҤᎡᑭ۞ᙯᔣЯ̄ 8, 10Ą

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Dermatol Sinica, December 2003

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Cutaneous Hodgkin's disease: an immunohistochemical analysis. J Cutan Pathol 22: 229-235, 1995. 16. Shulman LN, Frisard B, Antin JH, et al.: Primary Ki-1 large cell lymphoma in adult: Clinical characteristic and therapeutic outcome. J Clin Oncol 11: 937-942, 1993.

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