who-eortc classification for cutaneous lymphomas - Dermatology

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Immunophenotype The neoplastic cells express CD2, CD56, cytoplasmic CD3ε and cytotoxic proteins (TIA-1, granzyme B, perforin), but lack surface CD3. 122 In rare CD56 negative cases detection of EBV by in-situ- hybridization and expression of cytotoxic proteins are required for diagnosis. 3 (Fig. 5B). LMP-1 is inconsistently expressed. . Genetic features The T-cell receptor is usually in germ line configuration, but can be rearranged in rare tumors with a cytotoxic T-cell phenotype. EBV is expressed almost in all cases, suggesting a pathogenetic role of this virus. 18 Prognosis and predictive factors Nasal type NK/T-cell lymphoma presenting in the skin is a highly aggressive tumour with a median survival of less than 12 months. 18,19,120,121 The most important factor predicting poor outcome is the presence of extracutaneous involvement at presentation. In patients presenting with only skin lesions a median survival of 27 months was reported, compared to 5 months for patients presenting with cutaneous and extracutaneous disease. 121 CD30+, CD56+ cases reported to have a better prognosis may have been examples of C-ALCL with co-expression of CD56. 20 Therapy Systemic chemotherapy is the first choice of treatment, but the results are disappointing. 120,121 Variant Hydroa vacciniforme-like CTCL is a rare type of EBV-associated lymphoma of CD8+ cytotoxic T cells, which affects children almost exclusively in Latin America and Asia. 123-125 Patients present with a papulovesicular eruption clinically resembling hydroa vacciniforme, particularly on the face and upper extremities (sunexposed areas). The prognosis is poor. PRIMARY CUTANEOUS PERIPHERAL T-CELL LYMPHOMA, UNSPECIFIED PTL, unspecified in the WHO classification represent a heterogeneous group which includes all T-cell neoplasms that do not fit into any of the better defined subtypes of T-cell lymphoma/leukemia. Recent studies have suggested that primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, cutaneous gamma-delta T-cell lymphoma, and primary cutaneous small-medium CD4+ Tcell lymphoma can be separated out as provisional entities. For the remaining diseases that do not fit 20
into either of these provisional entities the designation PTL, unspecified, is maintained. In all cases a diagnosis of MF must be ruled out by complete clinical examination and an accurate clinical history. Primary cutaneous aggressive epidermotropic CD8-positive cytotoxic T-cell lymphoma (provisional entity) Definition CTCL characterized by a proliferation of epidermotropic CD8-positive cytotoxic T-cells and an aggressive clinical behavior. 25,26 Differentiation from other types of CTCL expressing a CD8-positive cytotoxic T-cell phenotype, as observed in more than 50% of patients with pagetoid reticulosis, and rare cases of MF, LyP, and C-ALCL, is based on the clinical presentation and clinical behavior. 25 In these latter conditions no difference in clinical presentation or prognosis between CD4+ and CD8+ cases is found. Clinical features Clinically, these lymphomas are characterized by the presence of localized or disseminated eruptive papules, nodules and tumors showing central ulceration and necrosis or by superficial, hyperkeratotic patches and plaques. 16, 25 (Fig. 6A). The clinical features are very similar to those observed in patients with a cutaneous γ/δ T-cell lymphoma and cases described as generalized pagetoid reticulosis (Ketron- Goodman type) in the past. 32 These lymphomas may disseminate to other visceral sites (lung, testis, central nervous system, oral mucosa), but lymph nodes are often spared. 25 Histopathology Histologically these lymphomas show an acanthotic or atrophic epidermis, necrotic keratinocytes, ulceration and variable spongiosis, sometimes with blister formation. 16,25 Epidermotropism is often pronounced ranging from a linear distribution to a pagetoid pattern throughout the epidermis (Fig. 6B). Invasion and destruction of adnexal skin structures are commonly seen. Angiocentricity and angioinvasion may be present. Tumour cells are small-medium or medium-large with pleomorphic or blastic nuclei. Immunophenotype The tumor cell have a betaF1+, CD3+, CD8+, granzyme B+, perforin+, TIA-1+, CD45RA+, CD45RO- , CD2-, CD4-, CD5-, CD7-/+ phenotype. 11,16,25,26,32 (Fig. 6C and 6D). EBV is generally negative. 21
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: Treatment Patients have generally b
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 and 36: Table 1. WHO-EORTC classification o
Page 37 and 38: Table 3. Characteristic features of
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
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who-eortc classification for cutaneous lymphomas - Dermatology

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