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Intravascular lymphomatosis and intracerebral haemorrhage

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Abstract

Intravascular lymphomatosis (IVL) is a rare, malignant B- or T-cell lymphoma with remarkable affinity for the endothelial cells of small vessels, particularly within the skin and central nervous system. It is a disease that mimics several neurological disorders, particularly those of cerebrovascular ischemic origin. The prognosis is generally poor, with a rapidly fatal outcome. As a result the diagnosis is often made at post-mortem. We report a rare case of a 73-year-old patient with IVL complicated by intracerebral haemorrhage. In literature two cases of systemic IVL complicated by intracerebral haemorrhage have been reported, but they presented initially with a disseminated intravascular coagulation (DIC). This is the first case of brain IVL complicated by intracerebral haemorrhage not associated to DIC. Increasing awareness of this disease as a differential diagnosis to a common clinical presentation may lead to more opportunities to evaluate new diagnostic and treatment approaches.

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References

  1. Drobacheff C, Blanc D, Zultak M, Humbert P, Carbillet JP, Dupond JL, Laurent R (1989) Malignant angioendotheliomatosis reclassification as an angiotropic lymphoma. Int J Dermatol 28:454–456

    Article  CAS  PubMed  Google Scholar 

  2. Sheibani K, Battifora H, Winberg CD, Burke JS, Ben-Ezra J, Ellinger GM, Quigley NJ, Fernandez BB, Morrow D, Rappaport H (1986) Further evidence that ‘malignant angioendotheliomatosis’ is an angiotropic large-cell lymphoma. N Engl J Med 314:943–948

    Article  CAS  PubMed  Google Scholar 

  3. Lui PC, Wong GK, Poon WS, Tse GM (2003) Intravascular lymphomatosis. J Clin Pathol 56(6):468–470

    Article  CAS  PubMed  Google Scholar 

  4. Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD (1999) World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997. J Clin Oncol 17(12):3835–3849

    CAS  PubMed  Google Scholar 

  5. Tateyama H, Eimoto T, Tada T, Kamiya M, Fujiyoshi Y, Kajiura S (1991) Congenital angiotropic lymphoma (intravascular lymphomatosis) of the T-cell type. Cancer 67:2131–2136

    Google Scholar 

  6. Stroup RM, Sheibani K, Moncada A, Purdy LJ, Battifora H (1990) Angiotropic (intravascular) large cell lymphoma: a clinicopathological study of seven cases with unique clinical presentations. Cancer 66:1781–1788

    Article  CAS  PubMed  Google Scholar 

  7. Smadja D, Mas JL, Fallet-Bianco C, Meyniard O, Sicard D, de Recondo J, Rondot P (1991) Intravascular lymphomatosis (neoplastic angioendotheliosis) of the central nervous system: case report and literature review. J Neurooncol 11:171–180

    Google Scholar 

  8. Szots M, Bors L, Kálmán E, Szapáry L, Illés Z (2008) Intravascular lymphomatosis: diagnostic problems of a rare disease. J Neurol 255(10):1590–1592

    Article  CAS  PubMed  Google Scholar 

  9. Kanda M, Suzumiya J, Ohshima K, Tamura K, Kikuchi M (1999) Intravascular large cell lymphoma: clinicopathological, immunohistochemical and molecular genetic studies. Leuk Lymphoma 34:569–580

    CAS  PubMed  Google Scholar 

  10. Stahl RL, Chan W, Duncan A, Corley CC Jr (1991) Malignant angioendotheliomatosis presenting as disseminated intravascular coagulopathy. Cancer 68:2319–2323

    Article  CAS  PubMed  Google Scholar 

  11. Ferreri AJ, Campo E, Seymour JF et al (2004) Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series 38 cases, with special emphasis on the “cutaneous variant”. Br J Hermatol 127(2):173–183

    Article  Google Scholar 

  12. Demirer T, Dail DH, Aboulafia DM (1994) Four varied cases of intravascular lymphomatosis and a literature review. Cancer 73(6):1738–1745

    Article  CAS  PubMed  Google Scholar 

  13. Murase T, Nakamura S, Kawauchi K, Matsuzaki H, Sakai C, Inaba T, Nasu K, Tashiro K, Suchi T, Saito H (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  CAS  PubMed  Google Scholar 

  14. Murase T, Nakamura S (1999) An Asian variant of intravascular lymphomatosis: an updated review of malignant histocytosis-like B-cell lymphoma. Leuk Lymphoma 33:459–473

    CAS  PubMed  Google Scholar 

  15. Wong P, Moonie A, Dennett X, Anpalahan M (2006) A case of intravascular lymphomatosis (IVL) presenting as polyneuropathy. Eur J Intern Med 17(1):59–60

    Article  PubMed  Google Scholar 

  16. Beristain X, Azzarelli B (2002) The neurological masquerade of intravascular lymphomatosis. Arch Neurol 59(3):439–443

    Article  PubMed  Google Scholar 

  17. Gaul C, Hanisch F, Neureiter D, Behrmann C, Neundörfer B, Winterholler M (2006) Intravascular lymphomatosis mimicking disseminated encephalomyelitis and encephalomyelopathy. Clin Neurol Neurosurg 108(5):486–489

    Article  PubMed  Google Scholar 

  18. Heinrich A, Vogelgesang S, Kirsch M, Khaw AV, Shinkeigaku Rinsho (2005) Intravascular lymphomatosis presenting as rapidly progressive dementia. Eur Neurol 54(1):55–58

    Article  PubMed  Google Scholar 

  19. Tsuji H, Mochiduki A, Hosaka A, Yoshizawa T, Tamaoka A (2008) Intravascular lymphomatosis manifesting clinically as subacute encephalopathy. Rinsho Shinkeigaku 48(5):328–332

    PubMed  Google Scholar 

  20. Liew CL, Shyu WC, Tsao WL, Li H (2006) Intravascular lymphomatosis mimicks a cerebral demyelinating disorder. Acta Neurol Taiwan 15(4):264–268

    PubMed  Google Scholar 

  21. Iijima M, Fujita A, Uchigata M, Katoo H (2007) Change of brain MRI findings in a patient with intravascular malignant lymphomatosis. Eur J Neurol 14(5):4–5

    Article  Google Scholar 

  22. Khalidi HS, Brynes RK, Browne P, Koo CH, Battifora H, Medeiros LJ (1998) Intravascular large B-cell lymphoma: the CD5 antigen is expressed by a subset of cases. Mod Pathol 10:983–988

    Google Scholar 

  23. Ponzoni M, Arrigoni G, Gould VE, Del Curto B, Maggioni M, Scapinello A, Paolino S, Cassisa A, Patriarca C (2000) Lack of CD29 (beta1 integrin) and CD54 (ICAM-1) adhesion molecules in intravascular lymphomatosis. Hum Pathol 31:220–226

    Article  CAS  PubMed  Google Scholar 

  24. DiGiuseppe JA, Nelson WG, Seifter EJ, Boitnott JK, Mann RB (1994) Intravascular lymphomatosis: a clinicopathologic study of 10 cases and assessment of response to chemotherapy. J Clin Oncol 12:2573–2579

    CAS  PubMed  Google Scholar 

  25. Ferreri AJ, Campo E, Ambrosetti A, Ilariucci F, Seymour JF, Willemze R, Arrigoni G, Rossi G, López-Guillermo A, Berti E, Eriksson M, Federico M, Cortelazzo S, Govi S, Frungillo N, Dell’Oro S, Lestani M, Asioli S, Pedrinis E, Ungari M, Motta T, Rossi R, Artusi T, Iuzzolino P, Zucca E, Cavalli F, Ponzoni M (2004) Anthracycline-based chemotherapy as primary treatment for intravascular lymphoma. Ann Oncol 15:1215–1221

    Article  CAS  PubMed  Google Scholar 

  26. Calamia KT, Miller A, Shuster EA, Perniciaro C, Menke DM (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

    CAS  PubMed  Google Scholar 

  27. Nakahara T, Saito T, Muroi A, Sugiura Y, Ogata M, Sugiyama Y, Yamamoto T (1999) Intravascular lymphomatosis presenting as an ascending cauda equina: conus medullaris syndrome: remission after biweekly CHOP therapy. J Neurol Neurosurg Psychiatry 67:403–406

    Article  CAS  PubMed  Google Scholar 

  28. Ferreri AJ, Dognini GP, Bairey O, Szomor A, Montalbán C, Horvath B, Demeter J, Uziel L, Soffietti R, Seymour JF, Ambrosetti A, Willemze R, Martelli M, Rossi G, Candoni A, De Renzo A, Doglioni C, Zucca E, Cavalli F, Ponzoni M (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(2):253–257

    Article  CAS  PubMed  Google Scholar 

  29. Sips GJ, Amory CF, Delman BN, Kleinman GM, Lipsey LR, Tuhrim S (2009) Intravascular lymphomatosis of the brain in a patient with myelodysplastic syndrome. Nat Rev Neurol 5(5):288–292

    Article  PubMed  Google Scholar 

  30. Koizumi M, Nishimura M, Yokota A, Munekata S, Kobayashi T, Saito Y (2001) Successful treatment of intravascular malignant lymphomatosis with high-dose chemotherapy and autologous peripheral blood stem cell transplantation. Bone Marrow Transplant 27:1101–1103

    Article  CAS  PubMed  Google Scholar 

  31. Yamaguchi M, Kimura M, Watanabe Y, Taniguchi M, Masuya M, Kageyama S, Katayama N, Ohno T, Kita K, Shiku H et al (2001) Successful autologous peripheral blood stem cell transplantation for relapsed intravascular lymphomatosis. Bone Marrow Transplant 27:89–91

    Article  CAS  PubMed  Google Scholar 

  32. Rosenberg RD, Aird WC (1999) Mechanism of disease: vascular-bed-specific haemostasis and hypercoagulable states. N Engl J Med 340:1555–1564

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Neurology, Department of Medicine, Bussolengo Hospital, Via Ospedale 2, 37012, Bussolengo, Verona, Italy

    Maria Grazia Passarin & Ebba Buffone

  2. Center for Neuro-Oncology, Dana-Farber/Brigham and Womens Cancer Center SW430D, 44 Binney St, Boston, MA, 02115, USA

    Patrick Y. Wen

  3. Division of Medical Oncology, Central Hospital, Bolzano, Italy

    Emanuela Vattemi

  4. Division of Pathology, Azienda Ospedaliera-Universitaria, Verona, Italy

    Claudio Ghimenton & Lamberto Bontempini

  5. Division of Neurology, Azienda Ospedaliera-Universitaria, Verona, Italy

    Sarah Ottaviani & Anna Maria Musso

  6. Division of Oncology, Spedali Civili, Brescia, Italy

    Rebecca Pedersini

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  1. Maria Grazia Passarin
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Correspondence to Maria Grazia Passarin.

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Passarin, M.G., Wen, P.Y., Vattemi, E. et al. Intravascular lymphomatosis and intracerebral haemorrhage. Neurol Sci 31, 793–797 (2010). https://doi.org/10.1007/s10072-010-0284-7

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  • DOI: https://doi.org/10.1007/s10072-010-0284-7

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