Lymphoma and plasma cell neoplasms
B cell lymphoma subtypes
Intravascular large B cell lymphoma

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 3 April 2017, last major update January 2011

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PubMed Search: intravascular large B cell lymphoma

Cite this page: Intravascular large B cell lymphoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/lymphomadiffuseintra.html. Accessed June 24th, 2017.
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
Microscopic (histologic) images

Images hosted on other servers:

1B: renal cell carcinoma; 1C: intravascular tumor; 1D: CD20+

Myeloperoxidase+ tumor


Renal tumor - fig. 1: intracapillary atypical mononuclear cells with irregular nuclei and occasional multiple nucleoli
fig. 2: mitotic figures; fig. 3A: CD20+; fig. 3B: CD3-; fig. 3C: BCL2+; fig. 3D: CD68-
fig. 4: EM shows foot process fusion and microvillous transformation and atypical lymphocytes distending a glomerular loop

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