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Complete surgical resection and/or radiation therapy are used to treat primary cutaneous anaplastic large cell lymphoma as a single lesion; the majority of patients experience total remission. [7] While radiation of the primary lesion and the surrounding lymph nodes has been advised for patients whose local lymph nodes are involved, adding ...
Upregulation of ALK is mainly due to chromosomal translocation t(2;17), resulting in a fusion gene of CLTC with ALK, [4] [7] but can rarely be due to t(2;5), fusing NPM1 with ALK; [2]: 378 the later is the usual finding in anaplastic large cell lymphoma (ALCL).
Unlike ALK-positive ALCL, ALK-negative ALCL tends to occur in older adults (median age at diagnosis: 55–60 years) and presents primarily with lymph node involvement; only 20% of patients with ALK-ALCL present with extra-nodal disease in sites such as the skin, bone, and soft tissues.
Anaplastic lymphoma kinase (ALK) was originally discovered in 1994 [5] [7] in anaplastic large-cell lymphoma (ALCL) cells. ALCL is caused by a (2;5)(p23:q35) chromosomal translocation that generates the fusion protein NPM-ALK, in which the kinase domain of ALK is fused to the amino-terminal part of the nucleophosmin (NPM) protein.
Primary cutaneous CD4-positive small/medium T-cell lymphoproliferative disorder Peripheral T-cell lymphoma, NOS Angioimmunoblastic T-cell lymphoma Follicular T-cell lymphoma Nodal peripheral T-cell lymphoma with T follicular helper phenotype Anaplastic large cell lymphoma, ALK-positive Anaplastic large cell lymphoma, ALK-negative
ALK-positive anaplastic large cell lymphoma: an aggressive, systemic ALCL that strongly expresses anaplastic lymphoma kinase, i.e. ALK. ALK-negative anaplastic large cell lymphoma: an aggressive, systemic ALCL that does not express ALK. Primary cutaneous anaplastic large cell lymphoma: a less aggressive ALCL that commonly presents as skin tumors.
Pfizer's lorlatinib was the first third-generation inhibitor and was approved in 2018 by the US FDA for ALK-positive NSCLC after progression on a first or second-generation inhibitor. Its macrocyclic structure was designed specifically to address some of the most recalcitrant resistance mutations.
Primary cutaneous CD30-positive T-cell lymphoproliferative disorders Primary cutaneous anaplastic large cell lymphoma; Lymphomatoid papulosis; Peripheral T-cell lymphoma not otherwise specified; Most common T cell lymphoma Variable, usually a mix small to large lymphoid cells with irregular nuclear contours CD3