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ALCL : Clinical Features

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Clinical Features: Primary systemic anaplastic large cell lymphoma (ALCL) tends to involve both lymph nodes and extranodal sites (skin, bone, soft tissues, lung, liver, and retina), regardless of the ALK status. Bone marrow is involved in about 10% of cases; however, CD30 positive cells are seen in as many as 30% of cases. Less commonly involved sites include gastrointestinal tract, mediastinum, and the central nervous system. Peripheral blood involvement with leukemic features may be seen in small cell variant of ALK+ ALCL.

The median age at diagnosis is 34 years for ALK-positive cases and 58 years for ALK-negative patients. Apart from age, there is no difference in the clinical presentation between ALK-positive and ALK-negative cases. Patients usually present in advanced stages (60% have stage III or IV disease) often with B symptoms, such as high-grade fever. There is peripheral and abdominal lymphadenopathy, and extranodal as well as bone marrow involvement. Skin lesions may be present.

Case History: Both this and the previous image are from the same case. The patient was a 40 y/o male with cheek mass. The tumor was positive for ALK, CD30, EMA, and CD45 (variable).

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