ALK-negative anaplastic large cell lymphoma (ALK(-) ALCL) is an uncommon CD30-positive T-cell lymphoma that presents a major diagnostic challenge. It affects individuals in a wide age range and a variety of nodal and extranodal sites. Both morphologically and immunohistochemically, anaplastic large cell lymphoma (ALCL) can mimic many other hematologic and non-hematologic malignancies. Lack of expression of pan T-cell markers and a spectrum of histologic appearances can be misleading. Recent research has shown that DUSP22 rearrangements occur in ALK(-) ALCL with a favorable prognosis. This article provides an overview of the many challenges associated with the diagnosis of this entity.
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