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Intravascular Large B-Cell Lymphoma

  • Amrita Goyal
  • Joi B. Carter
  • Judith A. Ferry
  • Lyn McDivitt DuncanEmail author

Abstract

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.

Keywords

Intravascular 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 

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Amrita Goyal
    • 1
  • Joi B. Carter
    • 2
  • Judith A. Ferry
    • 3
  • Lyn McDivitt Duncan
    • 4
    Email author
  1. 1.Harvard-MIT Division of Health Sciences and TechnologyHarvard Medical SchoolBostonUSA
  2. 2.Department of DermatologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  3. 3.Hematopathology Service, Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  4. 4.Dermatopathology Service, Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA

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