Intravascular large B-cell lymphoma with a fulminant clinical course: a case report with definite diagnosis post mortem

Ann Oncol. 2002 Sep;13(9):1503-6. doi: 10.1093/annonc/mdf214.


A patient is described who presented with pancytopenia, splenomegaly and excessively elevated lactate dehydrogenase levels in concurrence with signs of extramedullary hematopoiesis. Although initially considered in the differential diagnostic spectrum, a highly aggressive lymphoma could not be identified before the patient died, 6 weeks after admission. Even an intensive diagnostic work-up including splenectomy and repeated bone marrow biopsies was inconclusive. Finally, the diagnosis of an intravascular large B-cell lymphoma, a highly aggressive clinical subtype of a diffuse large B-cell lymphoma, spreading within vascular structures of multiple organs was established by autopsy. Intravascular large B-cell lymphoma is often not diagnosed before death due to the exclusive intravascular growth pattern of the tumor cells and a fulminant clinical course. The heterogeneous clinical features of this lymphoma subtype are discussed.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autopsy
  • Biopsy, Needle
  • Combined Modality Therapy
  • Disease Progression
  • Fatal Outcome
  • Humans
  • Immunohistochemistry
  • Lymphoma, B-Cell / complications
  • Lymphoma, B-Cell / pathology*
  • Lymphoma, B-Cell / therapy
  • Lymphoma, Large B-Cell, Diffuse / complications
  • Lymphoma, Large B-Cell, Diffuse / pathology*
  • Lymphoma, Large B-Cell, Diffuse / therapy
  • Male
  • Middle Aged
  • Severity of Illness Index
  • Splenectomy
  • Vascular Neoplasms / complications
  • Vascular Neoplasms / pathology*
  • Vascular Neoplasms / therapy