Article

Intravascular Large B-Cell Lymphoma

Department of Pathology, Medical University of South Carolina, Charleston, 29425, USA.
Archives of pathology & laboratory medicine (Impact Factor: 2.88). 03/2012; 136(3):333-8. DOI: 10.5858/arpa.2010-0747-RS
Source: PubMed

ABSTRACT A rare type of diffuse large B-cell lymphoma, intravascular large B-cell lymphoma primarily affects the middle-aged to elderly population, with a slight predominance in men. By the time of presentation, most patients have advanced, disseminated disease, and often the diagnosis is made at autopsy. Patients may present with any of a myriad of symptoms, with any tissue potentially being infiltrated. Central nervous system and cutaneous involvement is common, as is the presence of B symptoms including fever, weight loss, and night sweats. Morphologically, growth of neoplastic cells is restricted to the lumen of small vessels. The cells are large, with 1 or more prominent nucleoli, scant cytoplasm, and frequent mitotic figures, and are commonly positive for cluster of differentiation markers 79a, 20, and 19, as well as MUM1/IRF4 and Bcl-2. Intravascular large B-cell lymphoma is aggressive, and without treatment is rapidly fatal.

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    • "Two clinical variants associated with ethnogeographic origin have been described. Western patients usually present with the classical form, characterized by cutaneous involvement, as well as a higher propensity for CNS involvement; patients from Asian countries, on the other hand, tend to manifest hemophagocytic syndrome at diagnosis [7]. As is the case with DLBCL, CNS involvement portends an adverse prognosis with a median survival of less than 1 year. "
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