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Intravascular Large B-Cell Lymphoma

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Atlas of Cutaneous Lymphomas

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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.

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Authors and Affiliations

  1. Harvard-MIT Division of Health Sciences and Technology, Harvard Medical School, Boston, MA, USA

    Amrita Goyal M.D.

  2. Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Joi B. Carter M.D.

  3. Hematopathology Service, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Judith A. Ferry M.D.

  4. Dermatopathology Service, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Lyn McDivitt Duncan M.D.

Authors
  1. Amrita Goyal M.D.
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  2. Joi B. Carter M.D.
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  3. Judith A. Ferry M.D.
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  4. Lyn McDivitt Duncan M.D.
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Corresponding author

Correspondence to Lyn McDivitt Duncan M.D. .

Editor information

Editors and Affiliations

  1. Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts, USA

    Joi B. Carter

  2. Division of Health Sciences and Technology, Harvard Medical School, Boston, Massachusetts, USA

    Amrita Goyal

  3. Dermatopathology Service, Massachusetts General Hospital, Boston, Massachusetts, USA

    Lyn McDivitt Duncan

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© 2015 Springer International Publishing Switzerland

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Goyal, A., Carter, J.B., Ferry, J.A., Duncan, L.M. (2015). Intravascular Large B-Cell Lymphoma. In: Carter, J., Goyal, A., McDivitt Duncan, L. (eds) Atlas of Cutaneous Lymphomas. Springer, Cham. https://doi.org/10.1007/978-3-319-17217-0_19

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  • DOI: https://doi.org/10.1007/978-3-319-17217-0_19

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-17216-3

  • Online ISBN: 978-3-319-17217-0

  • eBook Packages: MedicineMedicine (R0)

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