Skip to main content
SpringerLink
Log in

Intravascular Lymphoma of the CNS

  • Chapter
  • First Online:
Lymphoma and Leukemia of the Nervous System

  • 971 Accesses

Abstract

Intravascular lymphoma is a rare form of non-Hodgkin lymphoma characterized by almost exclusive growth of neoplastic large lymphocytes within blood vessel lumina. Intravascular lymphoma is morphologically characterized in most instances by a B-cell phenotype, although rare cases with T- or NK-cell phenotype have been reported. This lymphoma is an aggressive and usually disseminated disease that predominantly affects elderly patients, resulting in poor performance status, B-symptoms, anemia, and a high serum lactate dehydrogenase level. The brain and skin are the most commonly involved sites. The usually disappointing survival after conventional chemotherapy has been recently improved by the introduction of immunotherapy (anti-CD20 antibody, rituximab). Differences in clinical presentation exist, mainly due to the association of IVL with the hemophagocytic syndrome. Intensive chemotherapy combinations containing drugs with higher central nervous system bioavailability are needed in cases with brain involvement.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Ponzoni M, Ferreri AJ, Campo E et al (2007) Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. J Clin Oncol 25:3168–3173

    Article  PubMed  Google Scholar 

  2. Pfleger L, Tappeiner J (1959) On the recognition of systematized endotheliomatosis of the cutaneous blood vessels (reticuloendotheliosis?). Hautarzt 10:359–363

    PubMed  CAS  Google Scholar 

  3. Sheibani K, Battifora H, Winberg CD et al (1986) Further evidence that “malignant angioendotheliomatosis” is an angiotropic large-cell lymphoma. N Engl J Med 314:943–948

    Article  PubMed  CAS  Google Scholar 

  4. Nakamura S, Ponzoni M, Campo E (2008) Intravascular large B-cell lymphoma. In: Swerdlow S, Campo E, Harris NL et al (eds) WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IARC, Lyon, pp 252–253

    Google Scholar 

  5. Baehring JM, Henchcliffe C, Ledezma CJ et al (2005) Intravascular lymphoma: magnetic resonance imaging correlates of disease dynamics within the central nervous system. J Neurol Neurosurg Psychiatry 76:540–544

    Article  PubMed  CAS  Google Scholar 

  6. Gill S, Melosky B, Haley L, ChanYan C (2003) Use of random skin biopsy to diagnose intravascular lymphoma presenting as fever of unknown origin. Am J Med 114:56–58

    Article  PubMed  Google Scholar 

  7. Ponzoni M, Ferreri AJ (2006) Intravascular lymphoma: a neoplasm of “homeless” lymphocytes? Hematol Oncol 24:105–112

    Article  PubMed  Google Scholar 

  8. Ferreri AJ, Campo E, Seymour JF et al (2004) Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the “cutaneous variant”. Br J Haematol 127:173–183

    Article  PubMed  Google Scholar 

  9. Kiyohara T, Kumakiri M, Kobayashi H et al (2000) A case of intravascular large B-cell lymphoma mimicking erythema nodosum: the importance of multiple skin biopsies. J Cutan Pathol 27:413–418

    Article  PubMed  CAS  Google Scholar 

  10. Ponzoni M, Arrigoni G, Gould VE et al (2000) Lack of CD 29 (beta1 integrin) and CD 54 (ICAM-1) adhesion molecules in intravascular lymphomatosis. Hum Pathol 31:220–226

    Article  PubMed  CAS  Google Scholar 

  11. Murase T, Nakamura S, Kawauchi K et al (2000) An Asian variant of intravascular large B-cell lymphoma: clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome. Br J Haematol 111:826–834

    Article  PubMed  CAS  Google Scholar 

  12. Ferreri AJ, Dognini GP, Campo E et al (2007) Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions. Haematologica 92:486–492

    Article  PubMed  Google Scholar 

  13. Takahashi E, Kajimoto K, Fukatsu T et al (2005) Intravascular large T-cell lymphoma: a case report of CD30-positive and ALK-negative anaplastic type with cytotoxic molecule expression. Virchows Arch 447:1000–1006

    Article  PubMed  Google Scholar 

  14. Yegappan S, Coupland R, Arber DA et al (2001) Angiotropic lymphoma: an immunophenotypically and clinically heterogeneous lymphoma. Mod Pathol 14:1147–1156

    Article  PubMed  CAS  Google Scholar 

  15. Murase T, Yamaguchi M, Suzuki R et al (2007) Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5. Blood 109:478–485

    Article  PubMed  CAS  Google Scholar 

  16. Williams G, Foyle A, White D et al (2005) Intravascular T-cell lymphoma with bowel involvement: case report and literature review. Am J Hematol 78:207–211

    Article  PubMed  Google Scholar 

  17. Malicki DM, Suh YK, Fuller GN, Shin SS (1999) Angiotropic (intravascular) large cell lymphoma of T-cell phenotype presenting as acute appendicitis in a patient with acquired immunodeficiency syndrome. Arch Pathol Lab Med 123:335–337

    PubMed  CAS  Google Scholar 

  18. Nakamichi N, Fukuhara S, Aozasa K, Morii E (2008) NK-cell intravascular lymphomatosis–a mini-review. Eur J Haematol 81:1–7

    Article  PubMed  Google Scholar 

  19. Conlin PA, Orden MB, Hough TR, Morgan DL (2001) Myeloperoxidase-positive intravascular large B-cell lymphoma. Arch Pathol Lab Med 125:948–950

    PubMed  CAS  Google Scholar 

  20. Seki K, Miyakoshi S, Lee GH et al (2004) Prostatic acid phosphatase is a possible tumor marker for intravascular large B-cell lymphoma. Am J Surg Pathol 28:1384–1388

    Article  PubMed  Google Scholar 

  21. Otrakji CL, Voigt W, Amador A et al (1988) Malignant angioendotheliomatosis–a true lymphoma: a case of intravascular malignant lymphomatosis studied by southern blot hybridization analysis. Hum Pathol 19:475–478

    Article  PubMed  CAS  Google Scholar 

  22. Sleater JP, Segal GH, Scott MD, Masih AS (1994) Intravascular (angiotropic) large cell lymphoma: determination of monoclonality by polymerase chain reaction on paraffin-embedded tissues. Mod Pathol 7:593–598

    PubMed  CAS  Google Scholar 

  23. Kanda M, Suzumiya J, Ohshima K et al (2001) Analysis of the immunoglobulin heavy chain gene variable region of intravascular large B-cell lymphoma. Virchows Arch 439:540–546

    Article  PubMed  CAS  Google Scholar 

  24. Vieites B, Fraga M, Lopez-Presas E et al (2005) Detection of t(14;18) translocation in a case of intravascular large B-cell lymphoma: a germinal centre cell origin in a subset of these lymphomas? Histopathology 46:466–468

    Article  PubMed  CAS  Google Scholar 

  25. Tsukadaira A, Okubo Y, Ogasawara H et al (2002) Chromosomal aberrations in intravascular lymphomatosis. Am J Clin Oncol 25:178–181

    Article  PubMed  Google Scholar 

  26. Kinoshita M, Izumoto S, Hashimoto N et al (2008) Immunohistochemical analysis of adhesion molecules and matrix metalloproteinases in malignant CNS lymphomas: a study comparing primary CNS malignant and CNS intravascular lymphomas. Brain Tumor Pathol 25:73–78

    Article  PubMed  Google Scholar 

  27. Ferry JA, Harris NL, Picker LJ et al (1988) Intravascular lymphomatosis (malignant angioendotheliomatosis). A B-cell neoplasm expressing surface homing receptors. Mod Pathol 1:444–452

    PubMed  CAS  Google Scholar 

  28. Murase T, Tashiro K, Suzuki T et al (1998) Detection of antibodies to Fasciola and Anisakis in Japanese patients with intravascular lymphomatosis. Blood 92:2182–2183

    PubMed  CAS  Google Scholar 

  29. Shimokawa I, Higami Y, Sakai H et al (1991) Intravascular malignant lymphomatosis: a case of T-cell lymphoma probably associated with human T-cell lymphotropic virus. Hum Pathol 22:200–202

    Article  PubMed  CAS  Google Scholar 

  30. Bergmann M, Terzija-Wessel U, Blasius S et al (1994) Intravascular lymphomatosis of the CNS: clinicopathologic study and search for expression of oncoproteins and Epstein-Barr virus. Clin Neurol Neurosurg 96:236–243

    Article  PubMed  CAS  Google Scholar 

  31. Au WY, Shek WH, Nicholls J et al (1997) T-cell intravascular lymphomatosis (angiotropic large cell lymphoma): association with Epstein-Barr viral infection. Histopathology 31:563–567

    Article  PubMed  CAS  Google Scholar 

  32. Song DE, Lee M-W, Ryu M-H et al (2007) Intravascular Large Cell Lymphoma of the Natural Killer Cell Type. J Clin Oncol 25:1279–1282

    Article  PubMed  CAS  Google Scholar 

  33. Cerroni L, Massone C, Kutzner H et al (2008) Intravascular large T-cell or NK-cell lymphoma: a rare variant of intravascular large cell lymphoma with frequent cytotoxic phenotype and association with Epstein-Barr virus infection. Am J Surg Pathol 32:891–898

    Article  PubMed  Google Scholar 

  34. Hsiao CH, Su IJ, Hsieh SW et al (1999) Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi’s sarcoma in an AIDS patient. Am J Surg Pathol 23:482–487

    Article  PubMed  CAS  Google Scholar 

  35. Nixon BK, Kussick SJ, Carlon MJ, Rubin BP (2005) Intravascular large B-cell lymphoma involving hemangiomas: an unusual presentation of a rare neoplasm. Mod Pathol 18:1121–1126

    Article  PubMed  CAS  Google Scholar 

  36. Ferry JA, Sohani AR, Longtine JA et al (2009) HHV8-positive, EBV-positive Hodgkin lymphoma-like large B-cell lymphoma and HHV8-positive intravascular large B-cell lymphoma. Mod Pathol 22:618–626

    Article  PubMed  Google Scholar 

  37. Shimada K, Kinoshita T, Naoe T, Nakamura S (2009) Presentation and management of intravascular large B-cell lymphoma. Lancet Oncol 10:895–902

    Article  PubMed  Google Scholar 

  38. Glass J, Hochberg FH, Miller DC (1993) Intravascular lymphomatosis. A systemic disease with neurologic manifestations. Cancer 15(71):3156–3164

    Article  Google Scholar 

  39. Beristain X, Azzarelli B (2002) The neurological masquerade of intravascular lymphomatosis. Arch Neurol 59:439–443

    Article  PubMed  Google Scholar 

  40. Calamia KT, Miller A, Shuster EA et al (1999) Intravascular lymphomatosis. A report of ten patients with central nervous system involvement and a review of the disease process. Adv Exp Med Biol 455:249–265

    PubMed  CAS  Google Scholar 

  41. Sienknecht CW, Whetsell WO Jr, Pollock P (1995) Intravascular malignant lymphoma (“malignant angioendotheliomatosis”) mimicking primary angiitis of the central nervous system. J Rheumatol 22:1769–1770

    PubMed  CAS  Google Scholar 

  42. Song DK, Boulis NM, McKeever PE, Quint DJ (2002) Angiotropic large cell lymphoma with imaging characteristics of CNS vasculitis. AJNR Am J Neuroradiol 23:239–242

    PubMed  Google Scholar 

  43. Hashimoto H, Naritomi H, Kazui S et al (1998) Presymptomatic brain lesions on MRI in a patient with intravascular malignant lymphomatosis. J Neuroimaging 8:110–113

    PubMed  CAS  Google Scholar 

  44. Schonfeld SM, Pinto RS, Aleksic S, Pearson J (1985 May) Cerebral angioendotheliomatosis: a report of two cases and review of the literature. AJNR Am J Neuroradiol 6:437–441

    PubMed  CAS  Google Scholar 

  45. Kenéz J, Barsi P, Majtényi K et al (2000) Can intravascular lymphomatosis mimic sinus thrombosis? A case report with 8 months” follow-up and fatal outcome. Neuroradiology 42:436–440

    Article  PubMed  Google Scholar 

  46. Oei ME, Kraft GH, Sarnat HB (2002) Intravascular lymphomatosis. Muscle Nerve 25:742–746

    Article  PubMed  Google Scholar 

  47. Levin KH, Lutz G (1996) Angiotropic large-cell lymphoma with peripheral nerve and skeletal muscle involvement: early diagnosis and treatment. Neurology 47:1009–1011

    PubMed  CAS  Google Scholar 

  48. Lacomis D, Smith TW, Long RR (1992) Angiotropic lymphoma (intravascular large cell lymphoma) presenting with cauda equina syndrome. Clin Neurol Neurosurg 94:311–315

    Article  PubMed  CAS  Google Scholar 

  49. Van Droogenbroeck J, Altintas S, Pollefliet C et al (2001) Intravascular large B-cell lymphoma or intravascular lymphomatosis: report of a case diagnosed by testicle biopsy. Ann Hematol 80:316–318

    Article  PubMed  Google Scholar 

  50. Kayano H, Katayama I (1990) Primary hepatic lymphoma presenting as intravascular lymphomatosis. Arch Pathol Lab Med 114:580–584

    PubMed  CAS  Google Scholar 

  51. Imai H, Kajimoto K, Taniwaki M et al (2004) Intravascular large B-cell lymphoma presenting with mass lesions in the central nervous system: a report of five cases. Pathol Int 54:231–236

    Article  PubMed  Google Scholar 

  52. O’Connor SR, Tan JH, Walewska R et al (2002) Angiotropic lymphoma occurring in a lacrimal sac oncocytoma. J Clin Pathol 55:787–788

    Article  PubMed  Google Scholar 

  53. Yamada N, Uchida R, Fuchida S et al (2005) CD5+ Epstein-Barr virus-positive intravascular large B-cell lymphoma in the uterus co-existing with huge myoma. Am J Hematol 78:221–224

    Article  PubMed  Google Scholar 

  54. Nakamura S, Murase T, Kinoshita T (2007) Intravascular large B-cell lymphoma: the heterogeneous clinical manifestations of its classical and hemophagocytosis-related forms. Haematologica 92:434–436

    Article  PubMed  Google Scholar 

  55. Ferreri AJ, Campo E, Ambrosetti A et al (2004) Anthracycline-based chemotherapy as primary treatment for intravascular lymphoma. Ann Oncol 15:1215–1221

    Article  PubMed  CAS  Google Scholar 

  56. Shimada K, Kosugi H, Narimatsu H et al (2008) Sustained remission after rituximab-containing chemotherapy for intravascular large B-cell lymphoma. J Clin Exp Hematop 48:25–28

    Article  PubMed  Google Scholar 

  57. Shimada K, Matsue K, Yamamoto K et al (2008) Retrospective analysis of intravascular large B-cell lymphoma treated with rituximab-containing chemotherapy as reported by the IVL study group in Japan. J Clin Oncol 26:3189–3195

    Article  PubMed  CAS  Google Scholar 

  58. Ferreri AJ, Dognini GP, Bairey O et al (2008) The addition of rituximab to anthracycline-based chemotherapy significantly improves outcome in “Western” patients with intravascular large B-cell lymphoma. Br J Haematol 143:253–257

    Article  PubMed  CAS  Google Scholar 

  59. Ferreri AJ, Dognini GP, Govi S et al (2008) Can rituximab change the usually dismal prognosis of patients with intravascular large B-cell lymphoma? J Clin Oncol 26:5134–5136; author reply 5136–5137

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Pathology Unit, Unit of Lymphoid Malignancies, Scientific Institute, San Raffaele Hospital, Milan, Italy

    Maurilio Ponzoni

  2. Departments of Neurology and Neurological Surgery, Thomas Jefferson University, Philadelphia, PA, USA

    Jon Glass

  3. Lymphoma Unit, IOSI – Oncology Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland

    Emanuele Zucca

Authors
  1. Maurilio Ponzoni
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jon Glass
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Emanuele Zucca
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Maurilio Ponzoni .

Editor information

Editors and Affiliations

  1. , Stephen E. and Catherine Pappas Center f, Massachusetts General Hospital, Fruit St. 55, Boston, 02114, Massachusetts, USA

    Tracy Batchelor

  2. Dept. Neurology, Memorial Sloan-Kettering Cancer Center, York Ave. 1275, New York, 10021-6007, New York, USA

    Lisa M. DeAngelis

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Ponzoni, M., Glass, J., Zucca, E. (2012). Intravascular Lymphoma of the CNS. In: Batchelor, T., DeAngelis, L. (eds) Lymphoma and Leukemia of the Nervous System. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-7668-0_14

Download citation

Publish with us

Policies and ethics

Search

Navigation