Intravascular Large B-Cell Lymphoma
Intravascular large B-cell lymphoma (ivLBCL) is an aggressive form of diffuse large B-cell lymphoma with a markedly poor prognosis. It commonly presents with central nervous system (CNS) symptoms and a wide variety of cutaneous manifestations, including maculopapular eruptions, ulcerated nodules, and infiltrated indurated plaques. Because the tumor cells lodge in the vasculature, in cases without obvious cutaneous disease a biopsy of a small cutaneous hemangioma may yield the diagnosis. ivLBCL has had numerous names in the literature, including angiotropic lymphoma, malignant angioendotheliomatosis, and angioendotheliomatosis proliferans systemisata. These names reflect the incorrect initial belief that the neoplastic cells were of endothelial origin; they are in fact derived from post–germinal center B cells [1, 2]. This chapter discusses the clinical presentation, prognosis, treatment, histopathology, immunohistochemistry, molecular characteristics, and differential diagnosis of iVLBCL. The chapter closes with two clinical cases featuring patients with ivLBCL.
KeywordsIntravascular large B-cell lymphoma Diffuse large B-cell lymphoma Post-germinal center B cells Hemophagocytic syndrome Lymphomatoid granulomatosis Primary diffuse large B-cell lymphoma of the central nervous system Angiotropic Hemophagocytic syndrome Western variant Asian variant
- 1.Ferreri AJM, Campo E, Seymour JF, Willemze R, Ilariucci F, Ambrosetti A, et al. Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the “cutaneous variant”. Br J Haematol. 2004;127:173–83.PubMedCrossRefGoogle Scholar
- 3.Nakamura S, Ponzoni M, Campo E. Intravascular large B-cell lymphoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri S, Stein H, et al., editors. WHO classification of tumors of haematopoietic and lymphoid tissues. Lyon: International Agency for Research On Cancer; 2008. p. 252–3.Google Scholar
- 17.Deisch J, Fuda FB, Chen W, Karandikar N, Arbini AA, Zhou XJ, et al. Segmental tandem triplication of the MLL gene in an intravascular large B-cell lymphoma with multisystem involvement: a comprehensive morphologic, immunophenotypic, cytogenetic, and molecular cytogenetic antemortem study. Arch Pathol Lab Med. 2009;133:1477–82.PubMedGoogle Scholar
- 24.Song DK, Boulis NM, Mckeever PE, Quint DJ. Angiotropic large cell lymphoma with imaging characteristics of CNS vasculitis. 2002;23:239–42.Google Scholar