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Primary effusion lymphoma presenting as a cutaneous intravascular lymphoma
Author(s) -
Crane Genevieve M.,
Xian Rena R.,
Burns Kathleen H.,
Borowitz Michael J.,
Duffield Amy S.,
Taube Janis M.
Publication year - 2014
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.12405
Subject(s) - primary effusion lymphoma , medicine , pathology , lymphoma , immunosuppression , skin biopsy , sarcoma , pleural effusion , context (archaeology) , biopsy , immunology , biology , paleontology
Primary effusion lymphoma ( PEL ) is a rare and aggressive lymphoma that arises in the context of immunosuppression and is characterized by co‐infection with Epstein–Barr virus ( EBV ) and human herpesvirus‐8/Kaposi sarcoma‐associated herpesvirus ( HHV ‐8/ KSHV ). It was originally described as arising in body cavity effusions, but presentation as a mass lesion (extracavitary PEL ) is now recognized. Here, we describe a case of PEL with an initial presentation as an intravascular lymphoma with associated skin lesions. The patient was a 53‐year‐old man with human immunodeficiency virus ( HIV )/acquired immune deficiency syndrome ( AIDS ) who presented with fevers, weight loss and skin lesions concerning for Kaposi sarcoma ( KS ). A skin biopsy revealed no evidence of KS ; however, dermal vessels contained large atypical cells that expressed CD31 and plasma cell markers but lacked most B‐ and T‐cell antigens. The atypical cells expressed EBV and HHV ‐8. The patient subsequently developed a malignant pleural effusion containing the same neoplastic cell population. The findings in this case highlight the potential for unusual intravascular presentations of PEL in the skin as well as the importance of pursuing microscopic diagnosis of skin lesions in immunosuppressed patients.