Transcription of 悪性リンパ腫 1．悪性リンパ腫の診断法 悪性リンパ腫はリン …
1 1 .. T B .. 1 .. 2 .. 1 .. 1) . T . B .. G .. B T . T .. 3 B DLBCL .. 2 . I IV .. CT .. PET-CT.. CT MRI .. 2 . (HL) (NHL) 2 . 4 . Reed-Sternberg R-S .. R-S CD15 CD30 .. B T . B CD19 CD20 . T CD2 CD3 CD5 CD4 CD8 .. B (DLBCL) . MALT . MALT .. B CD10 . t 14 ; 18 . bcl2 .. Burkitt t 8 ;. 14 c-myc . C-MYC BCL2 BCL6 . BCL10 DHL . HTLV-I T / . adult T cell leukemia/lymphoma ATL .. 4 .. 1 (WHO 4 ). (NHL). (HL). 2013 . 2 (NHL) .. 3 .. (1) B (DLBCL). DLBCL B CD20 95% . CD20 CD20 DLBCL . 5 ( ) CD20(B ) .. 6 ( ) . ADCC(NK ) ( ) CDC CD20 B .. 3 DLBCL R-CHOP .. 7 IPI ( R ) CHOP vs R-CHOP. 8 R-IPI R-CHOP .. R-IPI . DLBCL . (61 ). ( ). PS2 ( 50% ). 2 . LDH ( ). 5 Rivesed-International Prognostic Index . 9 (R-IPI) . 20 19 0 Very good(5 95%). 5 4 1-2 Good (5 80%). 2 1 3-5 Poor(5 55%).. NCCN-IPI R-CHOP R-IPI .. NCCN-IPI 2013.
2 4 10 NCNN-IPI. Monthly Hema Topic 2014 1 . CD20 R-CHOP . R-CHOP .. DLBCL . 11 DLBCL . 2013 . 12 DLBCL . 2013 . 13 DLBCL . 2013 .. (gemcitabine) . 2' 2 . Gemzar GEM .. DNA . C DNA . DNA DNA . DNA . DNA .. DLBCL 7 . 14 CD5 DLBCL .. 15 EB DLBCL EBV DLBCL.. 16 EBV DLBCL . 17 EBV DLBCL . 5 . DLBCL. (primary central nervous system lymphoma;. PCNSL) . 50 80 . high-dose methotrexate(HD-MTX) . (whole brain radiotherapy; WBRT) . PCNSL HD-MTX. ( g/m2) ( 30 Gy/15 17 ). 80 90% MST 40 .. acquired immune deficiency syndrome (AIDS) .. 60 .. 6 378 .. 18 DLBCL .. 19 DLBCL IELSG sore . 7 CNS DLBCL (IELSG sore). PS( ) LDH 5 . 2 (IELSG sore). 21 DLBCL . DLBXL CNS 7 (BJH, 2012, 159, 39 49) 60 LDH IPI . PS2 . DLBCL . 22 DLBCL . DLBCL. 1 2% 4% 1 9% . DLBCL .. 23 DLBCL R-CHOP . 24 DLBCL 3 1 10 . DLBCL.. 5% . DLBCL. 18-fluoro-deoxyglucose-positron emission tomography FDG-PET CT.
3 PET CT FDG-PET/CT . FDG-PET .. 2007 . DLBCL Hodgkin . FDG-PET CR . 25 Ga-SPECT PET-CT .. CD20 DLBCL . 26 CD20 CD19 DLBCL . DLBCL . 27 (FL) B (DLBCL) .. double-hit lymphoma (DHL). MYC BCL2 DLBCL DLBCL 5 % . WHO 4 (2008 ) MYC rearrangement DLBCL. "B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma" . "double-hit" . DLBCL MYC BCL2 10 % 20-30 % .. 28 (DHL) . 29 (DHL) . 31 (DHL) DLBCL . 32 DHL BCL6/MYC . DLBCL(IVL). 2 . Western form (classical IVLBL) - .. Asian Variant - . [5] . Western, Asian .. ( ) .. ( . ) . ( ) . Asian variant .. FDG-PET . B . (R-CHOP) . 8 Asian variant of intravascular large B-cell lymphoma(AIVL) .. 9 IVL Asian variant(AIVL) Vlassical IVL .. 33 Asian variant of intravascular large B-cell lymphoma(AIVL) . 10 (HPS) (Imajyuku ).
4 11 (HPS) . (Tsuda ).. 12 (LHPS) (Takahashi, Miura ).. 13 (HPS) .. 34 (HPS) .. 35 (HPS) .. 14 (LHPS) .. 36 (HPS) .. 15 (HPS) .. 16 (HPS) .. 2 &(MCL). B CD5 CD19 CD20 CD23 . t 11 ; 14 bcl1 cyclin D1.. 37 (MCL) . 2013 . 38 (MCL) . 2013 . 39 (MCL) .. (3) (FL).. III/IV 80 .. 10 15 . 60 30 40 .. 15 40% DLBCL . 40 (FL) . 2013 . 41 FL . 2013 . (3) MALT (MALT). NHL 9% DLBCL .. B . NF-kappa B .. 1. HCV. 2.. 3.. 4.. 5. ( ).. MALT . 10-30% t(11;18)(q21;q21) sub-population .. 42 MALT (MALT) . 43 MALT (MALT) . 2013 . 44 MALT (MALT). 2013 . 45 MALT (MALT) . 2013 . 46 MALT (MALT) . 2013 . (LPL). B B .. B . IgM M 3g/dl . IgM M.. WM / LPL MYD88L265P 90 100 . MYD88 Toll-like/ 1 . IRAK4. (interleukin-1 receptor-associated kinase4 IRAK1 . TRAF6 NFkB 23 25) ABC-DLBCL . 30 40 L265P GCB 10 .. lymphoplasmacytic lymphoma LPL.
5 Waldenstrom Waldenstrom's marcroglobulinemia WM . WHO WM .. 15-20 15 . LPL/WM B .. IgM IgD . CD19 . CD20 CD22 CD79 B CD11c CD25 . FMC7 CD5 CD23 10 20 .. CD138 CD20 .. Dutcher body PAS . B . LPL/WM 6 . 6q 6q- . IgM 3g/dl 10- 30 .. M . IgM . IgM 3000 mg/dl 10 . IgM monoclonal gammapathy of undetermined significance MGUS MGUS . 1-5 LPL/WM 5 . 10-30 . M .. M .. international prognostic scoring system . 10 2 3 g/ml IgM 7,000 mg/dl . 5 0 1 87 2 68 3-5 36 .. Ghobrial IM, et al. Lance Oncol 4: 679, 2003 .. M International Workshop(2006) Kimby E, et al. Clin Lymphoma Myeloma 6: 380, 2006 . 47 (LPL/WM) . 2013 .. c-myc B . WHO 4 [1] . BL .. endemic BL - . 5 70% .. sporadic BL - . BL(immunodeficiency associated BL) - HIV .. 1 2% [4] . 40 50% . CHOP . ( 10% ) .. modified CODOX-M/IVAC . R-Hyper-CVAD [13].. (40 ) 10cm LDH.
6 +7q del(13) . 48 . 2013 . 49 DLBCL . 2013 . 50 T .. (3 T PTCL(NOS).. T NK .. B .. PNP T .. DLBCL CHOP . PNP . FORODESINE T NK.. 51 T (PTCL) . 2013 . (4) (ALCL). T null cell type .. CD30 t 2 ; 5 . ALK .. 52 ALK ALCL .. MMAE CD30 .. 53 MMAE CD30 .. 17 MMAE CD30 . 18 MMAE CD30 .. 19 MMAE CD30 .. 20 MMAE CD30 .. 21 MMAE CD30 .. 22 MMAE CD30 .. 54 MMAE CD30 .. 52 MMAE CD30 ALCL .. 53 MMAE CD30 ALCL .. 23 MMAE CD30 ALCL .. 24 MMAE CD30 ALCL .. 54 T (AITL) .. 3 T ATLL . [1] HTLV-I T . HTLV-I ATL. HTLV-I DNA .. [2]ATL .. ATL . [3] . ATL . 1 . HTLV-I 1,000 . 2,000 1 T .. 2 . ATLL HTLV-I 57 . HTLV-I 120 HTLV-I. ATLL .. 55 T ATLL .. 56 T ATLL .. 57 T ATLL .. 58 T ATLL .. 3 . / R . ATL CD3 . CD4 CD8 HTLV-I . HTLV-I DNA ATL . ATLL LDH .. 59 ATL .. 4 . ATLL 1 . 4,000/ R . 4,000/ R .. 25 ATL .. 5 . ATLL .. 60 ATL.)
7 2013 . 26 ATL .. CCR4 CCD4 ATL (90%) . CCR4 . CCR4 IL-4 IL-5 . CD4 2 T . T . ATL 90 PTCL . 30 40 . 61 ( ) CCR4 .. 27 (ATL ) .. NK . 62 NK nasal type . 2013 . 62 NK nasal type .. : Hodgkin's lymphoma; HL . (Hodgkin cell) . (Reed-Sternberg cell) . (Hodgkin's disease; HD) . 1832 Thomas Hodgkin .. WHO . 4 . (Classical Hodgkin's lymphoma : CHL). o (Lymphocyte rich : LR). o (Nodular sclerosis : NS). o (Mixed cellularity : MC). o (Lymphocyte depleted : LD). (Nodular lymphocyte predominant Hodgkin's lymphoma : NLPHL). 63 .. ( ). ABVD [ 1]4 . involved field radiation therapy; IFRT .. ( ).. ( ). ABVD 6 8 6 8 . B 4 . 6 6 8 .. ( ).. 64 ABVD .. 65 . 2013 . 65 . 66 .. 28 HL . 29 HL .. 67 HL .. ESHAP [4] .. 2014 1 (CD30 ) .. (SG035-0003 ) 75% . 4 .. B .. 68 CHL .. 30 MMAE CD30 .. 31 MMAE CD30 . 69 CHL MMAE CD30.
8 70 CHL MMAE CD30 .. 32 CHL MMAE CD30 .. 71.
