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Pregunta:

What is ALK+ anaplastic large cell lymphoma?

Autor: H K



Respuesta:

• CD30-positive mature T-cell lymphoma with aberrant expression of the Anaplastic Lymphoma Kinase (ALK) protein. • Chromosomal rearrangements that fuse the 3’ portion of ALK on chromosome 2p23 and the 5’ portion of a partner gene that provides the promoter and leads to constitutive expression of the chimeric oncoprotein, as well as to to constitutive activation of the kinase function. • There are over 20 partner genes involved in ALK rearrangements. The predominant fusion partner is NPM1, which is involved in t(2;5)(p23;q35) • Lymph nodes are commonly involved (90%), and extranodalinvolvement is frequent (60%) • Median age of 30 years, usually present with advanced stage disease (stage III or IV), and systemic symptom


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Autor

H K
H K