Intravascular Large B-Cell Lymphoma

Department of Pathology, Medical University of South Carolina, Charleston, 29425, USA.
Archives of pathology & laboratory medicine (Impact Factor: 2.84). 03/2012; 136(3):333-8. DOI: 10.5858/arpa.2010-0747-RS
Source: PubMed


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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    ABSTRACT: Second lymphoid neoplasms are an uncommon but recognized feature of non-Hodgkin's lymphomas, putatively arising secondary to common genetic or environmental risk factors. Previous limited evaluations of clonal relatedness between successive mature B-cell malignancies have yielded mixed results. We describe the case of a man with intravascular large B-cell lymphoma involving the central nervous system who went into clinical remission following immunochemotherapy and brain radiation, only to relapse 2 years later with a plasmacytoma of bone causing cauda equina syndrome. The plasmacytoma stained strongly for the cell cycle regulator cyclin D1 on immunohistochemistry, while the original intravascular large cell lymphoma was negative, a disparity providing no support for clonal identity between the 2 neoplasms. Continued efforts atcataloging and evaluating unique associations of B-cell malignancies are critical to improving understanding of overarching disease biology in B-cell malignancies.
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    ABSTRACT: Intravascular large B cell lymphoma (IVLBCL) is a rare, aggressive extranodal B cell lymphoma, classified as a subset of diffuse B cell lymphoma. IVLBCL typically occurs in elderly persons and the clinical heterogeneity of the condition makes the diagnosis elusive in most cases. Most of the reported cases have been in the Asian population with the majority of the cases being diagnosed postmortem. We present a unique case of IVLBCL in a 65-year-old Caucasian male who presented with fever of unknown origin.
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    ABSTRACT: Intravascular large B-cell lymphoma is a rare entity that usually presents in late stages with non-specific symptoms. We present a case of an incidentally discovered intravascular large B-cell lymphoma in a 78-year-old man who underwent colectomy for medically refractory pseudomembranous colitis. The malignant lymphocytes were preferentially localized to small colonic submucosal vasculature, without any evidence of an extravascular tumor mass. The gastrointestinal system is an exceeding rare initial diagnostic site for intravascular lymphoma, and presentation with pseudomembranous colitis has not been previously reported. We discuss the current definition of intravascular lymphoma, clinicopathological variants, differential diagnoses, as well as current therapy.
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