Rheumaderm pp 249-265 | Cite as

Intravascular Lymphomatosis

A Report of Ten Patients with Central Nervous System Involvement and a Review of the Disease Process
  • K. T. CalamiaEmail author
  • A. Miller
  • E. A. Shuster
  • C. Perniciaro
  • D. M. Menke
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 455)


The clinical, radiographic, and pathological findings in ten cases of intravascular lymphomatosis with central nervous system involvement seen at our institution over a 15- year period are presented. Nine patients presented with a subacute, progressive multifocal neurologic disorder. Most patients had fever, anemia, and elevation of the erythrocyte sedimentation rate. As the illness evolved, computerized tomography scanning and magnetic resonance imaging showed evidence of multifocal central nervous system disease. Angiography was nondiagnostic but suggested vasculitis in six cases. A response to empiric corticosteroid treatment was typical but usually transient. In six patients, the diagnosis was made antemortem by brain biopsy. The prognosis of patients was primarily dependent on early diagnosis and treatment, before massive central nervous system damage occurred. Treatment with chemotherapy, with or without radiotherapy, was associated with stabilization of the disease in three of five patients.


Central Nervous System Involvement Brain Biopsy Central Nervous System Vasculitis Primary Angiitis Intravascular Lymphoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer Science+Business Media New York 1999

Authors and Affiliations

  • K. T. Calamia
    • 1
    Email author
  • A. Miller
    • 2
  • E. A. Shuster
    • 3
  • C. Perniciaro
    • 4
  • D. M. Menke
    • 5
  1. 1.Department of RheumatologyMayo Clinic JacksonvilleJacksonvilleUSA
  2. 2.Resident in Internal MedicineMayo Clinic JacksonvilleJacksonvilleUSA
  3. 3.Department of NeurologyMayo Clinic JacksonvilleJacksonvilleUSA
  4. 4.Department of DermatologyMayo Clinic JacksonvilleJacksonvilleUSA
  5. 5.Department of PathologyMayo Clinic JacksonvilleJacksonvilleUSA

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