112 ( 1 2 3 200040) ( 1) 634 64 1 2010 1 8 ( ) 2009 12 12 12 0.5~4 cm 12 16 12 30 39.6 ( ) 37.5 100 7=93 93 7=? 0.6 1979 2009 9 5 mm 2009 11 19 ( ) 37.8 98 20 138/ 70 mmhg( 1 mmhg=0.133 kpa) 4 3 3 2 2 3 3 2 2 (+) ( ) 1/3 1008-0678(2012)01-0112-07 R733.4 R747.9 A, ( 1987-),,,,E-mail:zhtding@hotmail.com
113 ( 1) (+) 1. 2.94 10 9 /L( 4.5~11.0 10 9 /L), 4.84 10 12 /L( 3.5 5.0 10 12 /L) 121 10 9 /L( 100 300 10 9 /L) 125 g L -1 ( 110 150 g L -1 ) 81.1% ( 45.0% 70.0%) 2. 25 U L -1 ( <50 U L -1 ) 41 U L -1 ( <30 U L -1 ) 3. 2.6 mmol L -1 ( 2.5 7 mmol L -1 ) 14 µmol L -1 ( 44 88 µmol L -1 ) 0.141 mmol L -1 ( 0.100 0.420 mmol L -1 ) 4. 3.4 mmol L -1 ( 3.5 5.5 mmol L -1 ) 132 mmol L -1 ( 135 147 mmol L -1 ) 2.08 mmol L -1 ( 2.1 2.6 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 22.50 mg L -1 ( <3.25 8.20 mg L -1 ) IgG 13.00 g L -1 ( 7.00 16.00 g L -1 ) IgE <44.88 ng ml -1 ( 0.00 240.00 ng ml -1 ) C3 0.82 g L -1 ( 0.90 1.80 mg L -1 ) C4 0.24 g L -1 ( 0.10 0.40 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 12-5 14.78 U ml -1 ( <35 U ml -1 ) CA 19-9 8.48 U ml -1 ( <35 U ml -1 ) NSE<15.7 ng ml -1 ( 15.7 17.0 ng ml -1 ) 7. 647 U L -1 ( 106 211 U L -1 ) 8. (++) 12 10 6 /L ( 0 10 6 /L) 6 10 6 /L( 0 8 10 6 /L) 715 mg L -1 ( 150 450 mg L -1 ) 2.9 mmol L -1 ( 2.5 4.5 mmol L -1 ) 115 mmol L -1 ( 120 132 mmol L -1 ) ( ) 9. MRI (2010-01-13) 3 4 4 5 5 6 6 7 CT (2010-01-19) 1 B 1. 9 10 (+) (+) 2. 2 1. MUP NCV CMAP F 2. (SLE) SNAP (1982) [1]
114 NP= (No extract) Sm ( 6 ) 11 >0.5 g/24 h (<4 10 9 /L) ( 1.5 10 9 /L) 2 ( 100 10 9 /L) dsdna 4 4 SLE 10% SLE ( ) SLE SLE (tick) 17
115 15 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
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 ４００ Ｔａｂ ２ Ｓｋｉｎ ｐａｔｈｏｌｏｇｙ ＨＥ ４００ 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 Ｔａｂ ３ 图３ 免疫组化分析 Ａｎａｌｙｓｉｓ ｏｆ Ｉｍｍｕｎｏｈｉｓｔｏｃｈｅｍｉｃａｌ 注 A 为肿瘤细胞表达 LCA (leucocyte common a ntigen 白细胞共同抗原)阳性 (Envision 4 0 0 ) B 为肿瘤细胞表达 CD3 阳性(Envision 4 0 0 ) C 为肿瘤细胞表达 Ki -6 7 增殖指数为 4 0 % ( E nv i si o n 4 0 0 ) 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 4 0 0 ) 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, 4 0 0 ) 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)
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
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, :,2006:529-539. [ J ]., 2 0 0 8, 6 : 641-643 Burns TM, Schaublin GA, Dyck PJ. Vasculitic neuropathies[j]. Neurol Clin,2007,25:89-113,,. [ J ].,2008,16:520-525 Dispenzieri A. POEMS syndrome: 2011 update on diagnosis, riskstratification, and management[j]. Am J Hematol,2011,86:591-601 Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas[j]. Blood,2005,105:3768-3785 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,2004,103:216-221 Kelly JJ, Karcher DS. Lymphoma and peripheral neuropathy: a clinical review[j]. Muscle Nerve,2005,31:301-313 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, 2007,127:860-868 (2011-11-08 2011-12-20 )