anaplastic large cell lymphoma
an·a·plas·tic large cell lym·pho·ma
anaplastic large cell lymphoma
A primary non-B cell non-Hodgkin lymphoma, usually of T cell lineage, which is immunoreactive for CD30 and often ALK (anaplastic lymphoma kinase) and characterised by scattered to abundant “hallmark cells”—medium-sized cells with abundant cytoplasm and kidney-shaped nucleus, which tend to aggregate around blood vessels. ALCLs arise either as a primary neoplasm in a node or lymphoid tissue, soft tissue, lung, or skin, or secondary to the transformation of a lymphoproliferative disorder (e.g., mycosis fungoides), T-cell lymphoma, lymphomatoid papulosis or Hodgkin lymphoma.DiffDx Classical Hodgkin lymphoma.
Clinical findings Often a late-presenting lymphoma with “B symptoms” including night sweating and weight loss.
Molecular >90% have rearrangement of T cell receptor, chromosomal translocation involving the nucleophosmin gene on chromosome 5.
Management CHOP, radiation for bulky disease.