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Clinical manifestations, pathologic features, and diagnosis of systemic anaplastic large cell lymphoma

Arnold S Freedman, MD
Jon C Aster, MD
Section Editor
Andrew Lister, MD, FRCP, FRCPath, FRCR
Deputy Editor
Rebecca F Connor, MD


The peripheral T cell lymphomas (PTCLs) are a heterogeneous group of generally aggressive neoplasms that constitute less than 15 percent of all non-Hodgkin lymphomas in adults. Anaplastic large cell lymphoma, T/null cell type (ALCL) accounts for approximately 2 percent of adult non-Hodgkin lymphoma and is the second or third most common PTCL histology in adults depending on the series analyzed. (See "Classification of the hematopoietic neoplasms".)

Four distinct forms of ALCL are recognized based on clinical features and molecular characterization:

Primary systemic ALCL, anaplastic lymphoma kinase positive (ALK-positive ALCL)

Primary systemic ALCL, anaplastic lymphoma kinase negative (ALK-negative ALCL)

Breast implant-associated ALCL (BI-ALCL)


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Literature review current through: Sep 2016. | This topic last updated: Oct 7, 2016.
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