Intravascular lymphoma

Dr Daniel J Bell and Dr Bruno Di Muzio et al.

Intravascular lymphoma (IVL), also known as intravascular lymphomatosis, corresponds to a rare variant of extranodal diffuse large B cell lymphoma that affects small and medium-sized vessels and has no specific clinical or laboratory findings. CNS and skin manifestations are the most common forms, however, any organ may be involved 1,2,6.

Intravascular lymphoma usually affects elderly patients in their 6th to 7th decades of life, with a male-to-female ratio of 1:2 3

It has non-specific clinical features 3,4:

  • B symptoms, including fever of unknown origin
  • CNS involvement: encephalopathy, subacute progressive neurological deficits (depending on the location of vascular occlusions)
  • cutaneous involvement

If CNS involvement, a mild to moderate elevation of CSF protein is usually present 4

The reason why lymphoma cells tend to stay in the intravascular space in intravascular lymphoma is a consequence of the absence of CD29 (β1 integrin) and CD54 (ICAM-1) surface ligands, which probably disable them from diapedesis across the endothelium 2

The diagnosis of intravascular lymphoma is made postmortem in over 50% of cases 2.

If there is CNS involvement, angiography (DSA), CT and MRI often show evidence of multiple vascular occlusions and stroke as non-specific multifocal abnormalities 4-6

  • T2/FLAIR: hypersignal abnormalities in a dynamic pattern (resolution of some and the new appearance of others) 5
  • DWI/ADC: restriction areas in a dynamic pattern 5
  • T1 C+ (Gd): a persistent mass-like enhancement may be noted in proximity to the T2 or DWI changes. Different patterns of parenchymal and meningeal enhancement may be seen 5,6

Intravascular lymphoma usually has a rapidly fatal outcome, with patient overall survival lasting only a few months. Intravascular lymphoma is sensitive to systemic chemotherapy, however, the treatment still remains suboptimal due to the rarity of this disorder and the difficulty to establish a diagnosis in time 2

This condition was first described in Germany by L Pfleger and J Tappeiner in 1959 and designated as angioendotheliomatosis proliferans systemisata 4

Entities which promote multifocal brain lesions associated with a rapidly
progressive dementia should be considered as a differential diagnosis (e.g. stroke, primary angiitis of the CNS, infection and neoplasia) 4,5.

Article information

rID: 36986
Synonyms or Alternate Spellings:
  • Intravascular lymphomatosis
  • Angioendotheliomatosis proliferans systemisata
  • Tappeiner-Pfleger disease
  • Malignant angioendotheliomatosis
  • Angiotropic large cell lymphoma
  • Intravascular lymphomas
  • Intravascular lymphoma (IVL)

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Cases and figures

  • Case 1: axial FLAIR
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  • Case 1: sagittal FLAIR
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  • Case 2: T1FSC+
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