ALK-negative systemic intravascular anaplastic large cell lymphoma presenting in the skin.
Systemic cases of the CD30-positive T-cell neoplasm, anaplastic large cell lymphoma (ALCL), are typically anaplastic lymphoma kinase (ALK)-positive. The failure to express ALK protein has been shown to portend a worse prognosis. We describe a case of ALK-negative systemic ALCL that presented as a violaceous plaque on the scalp of a 79-year-old man. Interestingly, the neoplastic cells were confined largely within vascular spaces, a configuration that is exceedingly rare in the skin and is more typically seen with intravascular large B-cell lymphoma. In addition, bcl-2 immunohistochemical staining was strongly positive in this case, which may portend a more aggressive clinical course. To our knowledge, this report represents the first case of an ALK-negative ALCL to present intravascularly in the skin. Therefore, the recognition of systemic anaplastic T-cell lymphoma present within the intravascular spaces is important to avoid misdiagnosis.
Department of Dermatology, Stanford University, Stanford, CA 94305, USA.
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Rieger
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Polidore
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Warnke
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Kim
D000368:Aged; D000469:Alkaline Phosphatase / Q000378:metabolism; D001808:Blood Vessels / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D017728:Lymphoma, Large-Cell, Anaplastic / Q000201:enzymology / Q000473:pathology*; D008297:Male; D009389:Neovascularization, Pathologic / Q000201:enzymology; D012535:Scalp / Q000473:pathology; D012878:Skin Neoplasms / Q000098:blood supply / Q000201:enzymology / Q000473:pathology*
D000469:Alkaline Phosphatase