Novel Targeted Therapeutics for Peripheral T-Cell Lymphoma

摘要: The peripheral T-cell lymphomas (PTCL) are a group of aggressive mature and natural killer cell (NK) neoplasm’s that present with great morphological molecular heterogeneity (Anderson et al. 1998). They relatively rare diseases, constituting <15 % all NHL’s (Groves 2000). 2008 WHO classification recognizes over 20 sub-types NK-cell malignancies (WHO 2008). These listed in Table 1 along the annual incidence rates 3 year survival as reported SEER database (Seer Cancer Statistic 1975). most common histologies include: PTCL, not otherwise specified (PTCL-NOS); anaplastic large lymphoma (ALCL) angioimmunoblastic (AITL). Similar to B-cell neoplasm’s, can be broadly classified or indolent malignancies. include PTCL-NOS, hepatosplenic lymphoma, gamma/delta extranodal NK/T-cell nasal type lymphoma. PTCL-NOS is often viewed “wastebasket” category for those diseases do fit cleanly into other sub-types. Most cases PTCL lack distinct genetic biological alterations prognostic models have largely relied on clinical features simple factors such proliferation. Despite poor understanding pathogenesis these significant progress has been made many entities. For example, ALK positive considered disease entity which distinguished from provisional negative ALCL, due pathogenesis, younger age it presents better prognosis. Based recent publications ‘The International Lymphoma Project’, an international effort 22 centers worldwide collected data 1,314 NK -cell we now recognize ALCL former associated markedly inferior prognosis (Vose 2008) 2.