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Intravascular lymphomatosis of the brain in a patient with myelodysplastic syndrome

Nature Reviews Neurology volume 5, pages 288–292 (2009)Cite this article

Abstract

Background. A 77-year-old retired research pharmacologist with a long-standing history of anemia and a recent pathologically confirmed diagnosis of myelodysplastic syndrome was referred to a stroke unit for evaluation of slowly progressive cognitive deterioration, confusion and paroxysmal stroke-like episodes. A previous neurological work-up had revealed no noteworthy abnormalities except for chronic bilateral caudate infarctions seen on MRI and CT examinations of the brain.

Investigations. Physical examination, laboratory testing, brain MRI scanning, EEG, transesophageal echocardiography, cerebral angiography, CT scanning, and brain biopsy.

Diagnosis. Intravascular lymphomatosis of the brain.

Management. Combined chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) and rituximab.

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Figure 1: Pretreatment and posttreatment cerebral MRI scans in a patient with myelodysplastic syndrome and symptoms of cognitive deterioration, confusion and paroxysmal stroke-like episodes.
Figure 2: Angiogram from a patient with myelodysplastic syndrome and symptoms of cognitive deterioration, confusion and paroxysmal stroke-like episodes.
Figure 3: Brain biopsy findings from a patient with myelodysplastic syndrome and symptoms of cognitive deterioration, confusion and paroxysmal stroke-like episodes, which show the presence of atypical lymphoid cell clusters within the lumen of a cerebral blood vessel.

References

  1. Vardiman, J. W., Harris, N. L. & Brunning, R. D. The World Health Organization (WHO) classification of the myeloid neoplasms. Blood 100, 2292–2302 (2002.

    Article  CAS  Google Scholar 

  2. Ponzoni, M. & Ferreri, A. J. M. Intravascular lymphoma: a neoplasm of 'homeless' lymphocytes? Hematol. Oncol. 24, 105–112 (2006).

    Article  PubMed  Google Scholar 

  3. Ferreri, A. J. et al. 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 (2004).

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  5. Corey, S. J. et al. Myelodysplastic syndromes: the complexity of stem-cell diseases. Nat. Rev. Cancer 7, 118–129 (2007).

    Article  CAS  PubMed  Google Scholar 

  6. Strom, S. S., Gu, Y., Gruschkus, S. K., Pierce, S. A. & Estey, E. H. (2005). Risk factors of myelodysplastic syndromes: a case–control study. Leukemia 19, 1912–1918.

    Article  CAS  PubMed  Google Scholar 

  7. Pedersen-Bjergaard, J. Insights into leukemogenesis from therapy-related leukemia. N. Engl. J. Med. 352, 1591–1594 (2005).

    Article  CAS  PubMed  Google Scholar 

  8. Platzbecker, U., Meredyth-Stewart, M. & Ehninger, G. The pathogenesis of myelodysplastic syndromes (MDS). Cancer Treat. Rev. 33 (Suppl. 1), S53–S58 (2007).

    Article  CAS  Google Scholar 

  9. Nolte, F. & Hofmann, W. K. Myelodysplastic syndromes: molecular pathogenesis and genomic changes. Ann. Hematol. 87, 777–795 (2008).

    Article  PubMed  Google Scholar 

  10. Nimer, S. D. MDS: a stem cell disorder—but what exactly is wrong with the primitive hematopoietic cells in this disease? Hematology Am. Soc. Hematol. Educ. Program 2008 43–51 (2008).

    Article  Google Scholar 

  11. Amin, H. M. et al. Increased apoptosis in bone marrow B lymphocytes but not T lymphocytes in myelodysplastic syndrome. Blood 102, 1866–1868 (2003).

    Article  CAS  PubMed  Google Scholar 

  12. Culligan, D. J., Cachia, P., Whittaker, J., Jacobs, A. & Padua, R. A. Clonal lymphocytes are detectable in only some cases of MDS. Br. J. Haematol. 81, 346–352 (1992).

    Article  CAS  PubMed  Google Scholar 

  13. Katsarou, O. et al. Myelodysplastic features in patients with long-term HIV infection and haemophilia. Haemophilia 7, 47–52 (2001).

    Article  CAS  PubMed  Google Scholar 

  14. Lai, R., Arber, D. A., Brynes, R. K., Chan, O. & Chang, K. L. Untreated chronic lymphocytic leukemia concurrent with or followed by acute myelogenous leukemia or myelodysplastic syndrome: a report of five cases and review of the literature. Am. J. Clin. Pathol. 111, 373–378 (1999).

    Article  CAS  PubMed  Google Scholar 

  15. Gottardi, M. et al. Concomitant chronic lymphocytic leukemia and acute myeloid leukemia: evidence of simultaneous expansion of two independent clones. Leuk. Lymphoma 47, 885–889 (2006).

    Article  PubMed  Google Scholar 

  16. Svajdler, M., Lazúrová, I., Bohus, P. & Pal'ko, M. Intravascular variant of diffuse large B-cell lymphoma with combined endocrine involvement. Wien. Klin. Wochenschr. 118, 422–425 (2006).

    Article  PubMed  Google Scholar 

  17. Conlin, P. A., Orden, M. B., Hough, T. R. & Morgan, D. L. Myeloperoxidase-positive intravascular large B-cell lymphoma. Arch. Pathol. Lab. Med. 125, 948–950 (2001).

    CAS  PubMed  Google Scholar 

  18. Ozaki, S. et al. Myelodysplastic syndrome with neoplastic angioendotheliosis: report of a case [Japanese]. Rinsho Ketsueki 32, 1509–1514 (1991).

    CAS  PubMed  Google Scholar 

  19. Smith, M. T., Jones, R. M. & Smith, A. H. Benzene exposure and risk of non-Hodgkin lymphoma. Cancer Epidemiol. Biomarkers Prev. 16, 385–391 (2007).

    Article  CAS  PubMed  Google Scholar 

  20. Hou, Y. H. et al. Identification of a human B-cell/myeloid common progenitor by the absence of CXCR4. Blood 105, 3488–3492 (2005).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Graf, T. Differentiation plasticity of hematopoietic cells. Blood 99, 3089–3101 (2002).

    Article  CAS  PubMed  Google Scholar 

  22. Baehring, J. M., Longtine, J. & Hochberg, F. H. A new approach to the diagnosis and treatment of intravascular lymphoma. J. Neurooncol. 61, 237–248 (2003).

    Article  PubMed  Google Scholar 

  23. Koizumi, M. et al. Successful treatment of intravascular malignant lymphomatosis with high-dose chemotherapy and autologous peripheral blood stem cell transplantation. Bone Marrow Transplant. 27, 1101–1103 (2001).

    Article  CAS  PubMed  Google Scholar 

  24. Coiffier, B. et al. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N. Engl. J. Med. 346, 235–242 (2002).

    Article  CAS  PubMed  Google Scholar 

  25. Pfreundschuh, M. et al. CHOP-like chemotherapy plus rituximab versus CHOP-like chemotherapy alone in young patients with good-prognosis diffuse large-B-cell lymphoma: a randomised controlled trial by the MabThera International Trial (MInT) Group. Lancet Oncol. 7, 379–391 (2006).

    Article  CAS  PubMed  Google Scholar 

  26. Shimada, K. et al. 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 (2008).

    Article  CAS  PubMed  Google Scholar 

  27. Ferreri, A. J. et al. 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 (2008).

    Article  CAS  PubMed  Google Scholar 

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Acknowledgements

Written consent for publication was obtained from the patient. The authors would like to thank the patient described for his kind cooperation and permission to publish this case. Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. University Medical Center Groningen, Groningen, The Netherlands

    Gregorius J. Sips

  2. Mount Sinai Medical Center, NY, USA

    Colum F. Amory, Bradley N. Delman, George M. Kleinman, Lewis R. Lipsey & Stanley Tuhrim

Authors
  1. Gregorius J. Sips
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Correspondence to Stanley Tuhrim.

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The authors declare no competing financial interests.

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Sips, G., Amory, C., Delman, B. et al. Intravascular lymphomatosis of the brain in a patient with myelodysplastic syndrome. Nat Rev Neurol 5, 288–292 (2009). https://doi.org/10.1038/nrneurol.2009.36

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