who-eortc classification for cutaneous lymphomas - Dermatology

More documents

Recommendations

Info

Table 2. Relative frequency and disease-specific 5-year-survival of 1905 primary cutaneous lymphomas classified according to the WHO-EORTC classification WHO-EORTC Classification CUTANEOUS T-CELL LYMPHOMA Indolent clinical behaviour Mycosis fungoides Folliculotropic MF Pagetoid reticulosis Granulomatous slack skin Primary cutaneous anaplastic large cell lymphoma Lymphomatoid papulosis Subcutaneous panniculitis-like T-cell lymphoma Primary cutaneous CD4+ small/medium pleomorphic Tcell lymphoma * Aggressive clinical behaviour Sézary syndrome Primary cutaneous NK/T-cell lymphoma, nasal-type Primary cutaneous aggressive CD8+ T-cell lymphoma * Primary cutaneous γ/δ T-cell lymphoma * Primary cutaneous peripheral T-cell lymphoma, unspecified $ CUTANEOUS B-CELL LYMPHOMA Indelent clinical behaviour Primary cutaneous marginal zone B-cell lymphoma Primary cutaneous follicle center lymphoma Intermediate clinical behaviour Primary cutaneous diffuse large B-cell lymphoma, leg type Primary cutaneous diffuse large B-cell lymphoma, other Primary cutaneous intravascular large B-cell lymphoma Number 800 86 14 4 146 236 18 39 52 7 14 13 47 127 207 85 4 6 Frequency (%) # 44 4
Table 3. Characteristic features of PCFCL and PCLBCL, leg type PCFCL Morphology Predominance of centrocytes that are often large especially in diffuse lesions. Centroblasts may be present, but not in confluent sheets Growth pattern may be follicular, follicular and diffuse or diffuse (a continuum without distinct categories or grades). Phenotype Bcl-2: -/+ [1] Bcl-6: + CD10: -/+ [2] Mum-1: - Clinical Features Middle aged adults Localized lesions on head or trunk (90%) Multifocal lesions in rare cases PCLBCL, LEG TYPE Predominance or confluent sheets of medium-sized to large B-cells with round nuclei, prominent nucleoli and coarse chromatin resembling centroblasts and or immunoblastst Diffuse growth pattern. Bcl-2: ++ [3] Bcl-6: +/- CD10: - Mum-1: + Elderly, especially females Lesions localized on leg(s), most often below the knee. Rare cases with lesions at other sites than the leg (10%) [1]: faint staining, when present; minority of cells (see comments in paragraph on PCFCL) [2]: diffuse lesions mostly CD10 negative [3]: strong staining; in most neoplastic cells 37
Page 1 and 2: Blood First Edition Paper, prepubli
Page 3 and 4: Introduction A variety of T-and B-c
Page 5 and 6: Primary cutaneous follicle center c
Page 7 and 8: 1B). The presence of intraepidermal
Page 9 and 10: acneiform lesions, indurated plaque
Page 11 and 12: Clinical features This condition sh
Page 13 and 14: Extracorporeal photopheresis (ECP),
Page 15 and 16: Clinical features C-ALCL affects ma
Page 17 and 18: The large atypical cells in the LyP
Page 19 and 20: Treatment Patients have generally b
Page 21 and 22: into either of these provisional en
Page 23 and 24: Immunophenotype The tumor cells cha
Page 25 and 26: Clinical features Patients are comm
Page 27 and 28: Definition Primary cutaneous margin
Page 29 and 30: indurated plaques, which may preced
Page 31 and 32: that expression of bcl-2 protein by
Page 33 and 34: Clinically, they show similarities
Page 35: Table 1. WHO-EORTC classification o
Page 39 and 40: 10. Beljaards RC, Meijer CJLM, van
Page 41 and 42: 30. Jones D, Vega F, Sarris A, Mede
Page 43 and 44: 51. Smoller BR, Santucci M, Wood GS
Page 45 and 46: 73. Burns MK, Chan LS, Cooper KD. W
Page 47 and 48: 94. Lundin J, Hagberg H, Repp R. et
Page 49 and 50: 112. El Shabrawi-Caelen L, Kerl H,
Page 51 and 52: 133. Torne R, Su WPD, Winkelmann RK
Page 53 and 54: 154. Aarts WM, Willemze R, Bende RJ
Page 55 and 56: 175. Perniciaro C, Winkelmann RK, D
Page 57 and 58: Figure 6. Primary cutaneous CD4-pos
Page 59 and 60: fig 3 59
Page 61 and 62: fig 6 fig 7 fig 8 61

who-eortc classification for cutaneous lymphomas - Dermatology

You also want an ePaper? Increase the reach of your titles

Delete template?

Save as template?