Lymphoma & related disorders

Mature B cell neoplasms

Large B cell lymphomas-special subtypes

Intravascular



Topic Completed: 1 January 2011

Minor changes: 18 November 2021

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PubMed Search: intravascular large B cell lymphoma

Nikhil Sangle, M.D.
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Cite this page: Sangle N. Intravascular. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomadiffuseintra.html. Accessed December 6th, 2021.
Definition / general
  • A type of diffuse large B cell lymphoma with intravascular growth in skin, CNS, other sites; lymph nodes usually spared
Terminology
  • Previously called angiotropic lymphoma, but this is a confusing term
Clinical features
  • Rare
  • Median age 71 years, often fatal
  • Complex signs and symptoms, including fever of unknown origin, skin rash, mental status changes, rapidly progressive dementia
  • Frequent delay in diagnosis, leading to poor prognosis
  • Symptoms are related to the main organ involved
  • Often diagnosed at autopsy with involvement of multiple organs
  • May be a heterogeneous group of tumors, with some cases a transformation from other lymphomas (Mod Pathol 2001;14:1147)
Case reports
Treatment
  • Combination chemotherapy may be effective if detected early
Microscopic (histologic) description
  • Large centroblast-like lymphoid cells with prominent nucleoli within small vessel lumina (often capillaries) except in lymph nodes
  • Frequent mitotic figures; often fibrin thrombi
Positive stains
  • Similar to diffuse large B cell lymphoma (positive for CD19, CD20, CD22, CD79a), although rarely have T cell origin
  • CD10 (13% cases) and CD5 (38% cases)
  • CD10 negative cases are IRF4 / MUM1 positive
  • Usually BCL2 positive
Negative stains
Molecular / cytogenetics description
  • Often immunoglobulin gene rearrangement, rarely T cell receptor gene rearrangements
Differential diagnosis
  • Granulocytic sarcoma
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