Anaplastic large cell lymphoma, ALK-positive
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Note: This neoplasm has a small cell variant. (See below.)
Anaplastic large cell lymphoma, ALK-positive (ALCL, ALK+) frequently involves both lymph nodes and extranodal sites.The most commonly involved extranodal sites include skin, bone, soft tissue, lung, and liver. Involvement of the GI system and CNS is rare. Mediastinal disease is less frequent than in Classical Hodgkin lymphoma
Bone marrow involvement occurs in approximately 10-30% depending on the tests performed. The small cell variant may have a leukemic presentation with peripheral blood involvement.
Patients often have B-symptoms, especially high fever.
The immunophenotyping is vital in differentiating this neoplasm from large B-cell lymphoma with immunoblastic/plasmablastic features expressing ALK protein, rhabdomyosarcoma, and inflammatory myofibroblastic tumors.