113 ( 1) (+) /L( 4.5~ /L), /L( /L) /L( /L) 125 g L -1 ( g L -1 ) 81.1% (

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1 112 ( ) ( 1) ( ) ~4 cm ( ) = =? mm ( ) / 70 mmhg( 1 mmhg=0.133 kpa) (+) ( ) 1/ (2012) R733.4 R747.9 A, ( 1987-),,,, [email protected]

2 113 ( 1) (+) /L( 4.5~ /L), /L( /L) /L( /L) 125 g L -1 ( g L -1 ) 81.1% ( 45.0% 70.0%) U L -1 ( <50 U L -1 ) 41 U L -1 ( <30 U L -1 ) mmol L -1 ( mmol L -1 ) 14 µmol L -1 ( µmol L -1 ) mmol L -1 ( mmol L -1 ) mmol L -1 ( mmol L -1 ) 132 mmol L -1 ( mmol L -1 ) 2.08 mmol L -1 ( mmol L -1 ) 5. 1 DNA ( ) 4 mm h -1 ( 20 mm h -1 ) RF <10.30 U ml -1 ( <15.00 U ml -1 ) CRP mg L -1 ( < mg L -1 ) IgG g L -1 ( g L -1 ) IgE <44.88 ng ml -1 ( ng ml -1 ) C g L -1 ( mg L -1 ) C g L -1 ( g L -1 ) 6. AFP 3.31 µg L -1 ( <10 µg L -1 ) CEA 1.14 µg L -1 ( <10 µg L -1 ) CA U ml -1 ( <35 U ml -1 ) CA U ml -1 ( <35 U ml -1 ) NSE<15.7 ng ml -1 ( ng ml -1 ) U L -1 ( U L -1 ) 8. (++) /L ( /L) /L( /L) 715 mg L -1 ( mg L -1 ) 2.9 mmol L -1 ( mmol L -1 ) 115 mmol L -1 ( mmol L -1 ) ( ) 9. MRI ( ) CT ( ) 1 B (+) (+) MUP NCV CMAP F 2. (SLE) SNAP (1982) [1]

3 114 NP= (No extract) Sm ( 6 ) 11 >0.5 g/24 h (< /L) ( /L) 2 ( /L) dsdna 4 4 SLE 10% SLE ( ) SLE SLE (tick) 17

4 cm 10 1 M [2~4] [5] Crow-Fukasse POEMS 2 1 ( 3) POEMS POEMS (LDH) B 12 POEMS 50 Dispenzieri [5] POEMS 93% 3 31% 4 30% 5 ( 2 ( - ) POEMS POEMS ( ) ( 3) CD3(+) CD8( /+) KP1 ( ) CD138( ) CD1a( ) S-100( ) EBV (Epstein-Barr virus) ( ) EBER(+) (LDH) ( /+) LCA(+) TIA-1(+) B(+) L26( ) CD79a - (primary cutaneous lymphoma PCL) 2005 WHO

5 中国临床神经科学２０１２年第２０卷第１期 116 图２皮肤病理苏木精伊红染色４００Ｔａｂ２ＳｋｉｎｐａｔｈｏｌｏｇｙＨＥ４００注 A 肿瘤细胞浸润皮肤 B 肿瘤细胞以血管为中心浸润 C 凋亡小体 D 核异型箭头所指示核分裂象 E 凝固性坏死 Notes A. Tumor infiltrates the skin; B. Tumor infiltrates around the vessels; C. Apoptotic bodies; D. Neclear atypia; E. Coagulation necrosis Ｔａｂ３图３免疫组化分析ＡｎａｌｙｓｉｓｏｆＩｍｍｕｎｏｈｉｓｔｏｃｈｅｍｉｃａｌ注 A 为肿瘤细胞表达 LCA (leucocyte common a ntigen 白细胞共同抗原)阳性 (Envision ) B 为肿瘤细胞表达 CD3 阳性(Envision ) C 为肿瘤细胞表达 Ki -6 7 增殖指数为 4 0 % ( E nv i si o n ) D 为 E B E R 显示汗腺周围散在异型肿瘤细胞阳性阳性表达于细胞核( E B E R ) E 为 G B 显示汗腺周围散在肿瘤细胞阳性胞质内棕黄色颗粒状为阳性( G B, ) Notes A. The positive expression of LCA (leucocyte common antigen) in tumor cells(envision, 400); B. The positive expression of CD3(Envision, 400); C. Ki-67 proliferation index was 40%(Envision, 400); D. The scattered atypical tumor cells around the sweat glands were positive positioning in the nucleus(eber, 400); E. The scattered atypical tumor cells around the sweat glands were positive positioning in the cytoplasm(gb, 400)

6 117 (European Organization for Research and Treatment of Cancer EORTC) EBV ( EBER) WHO-EORTC ( 4) [6] /T (extranodal natural killer/ T-cell lymphoma EN-NK/TL) (cytotoxic granule protein) (nasal type) (nonnasal type) /T (extranodal natural kill (NK)/T-cell lymphoma, nasal type EN-NK/T-NT) NK EBV NK EBV NK EBV [7,8] EBV 5

7 118 ( ) ( / ) ( T ) / 635 (ACVBP) CHOP ACVBP 5 N K (hemophagocytic syndrome) EBV T ( - ) [1] ( ) ( ) CD2(+) CD56(+) CD3ε(+) CD3( ) CD5( ) CD4( ) CD8 ( ) EBV EBER(+) (granul associated protein) B(grazyme B) (+) ( ) (+) EBER(+) EN-NK/T-NT EN-NK/TL EN-NK/TL [9] (CHOP) T CHOP [2] [3] [4] [5] [6] [7] [8] [9],. : [ M ]. 2, :,2006: [ J ]., , 6 : Burns TM, Schaublin GA, Dyck PJ. Vasculitic neuropathies[j]. Neurol Clin,2007,25:89-113,,. [ J ].,2008,16: Dispenzieri A. POEMS syndrome: 2011 update on diagnosis, riskstratification, and management[j]. Am J Hematol,2011,86: Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas[j]. Blood,2005,105: Chim CS, Ma SY, Au WY, et al. Primary nasal natural killer cell lymphoma: long-term treatment outcome and relationship with the International Prognostic Index[J]. Blood,2004,103: Kelly JJ, Karcher DS. Lymphoma and peripheral neuropathy: a clinical review[j]. Muscle Nerve,2005,31: Hasserjian RP, Harris NL. NK-cell lymphomas and leukemias: a spectrum of t umors with vari able manifestat ions and immunophenotype[j]. Am J Clin Pathol, 2007,127: ( )

(CIP) /,,. :,2005 ( ) ISBN 7 81010 879 4................R72 CIP (2005) 076544 ( ht tp: / / www. tcmonline. com. cn) ( 1200 201203) 850 mm 1168 mm 1/ 3

(CIP) /,,. :,2005 ( ) ISBN 7 81010 879 4................R72 CIP (2005) 076544 ( ht tp: / / www. tcmonline. com. cn) ( 1200 201203) 850 mm 1168 mm 1/ 32 14 339 1 4 160 2005 11 1 2005 11 1 ISBN 7 81010 879