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Intravascular lymphoma associated with hemophagocytic syndrome: a rare but aggressive clinical entity

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Abstract

We report a 55-year-old male with a diagnosis of intravascular lymphoma and hemophagocytosis. He initially presented with hemolytic anemia and splenomegaly and was successfully treated with oral steroids. His clinical course was later complicated by fever, cytopenias, hypoalbuminemia, disseminated intravascular coagulation, gastrointestinal bleeding and acute tubular necrosis. Results of an extensive investigation for fever of unknown origin were negative. Although the patient was treated aggressively with antimicrobials, transfusion support and dialysis, he expired 3 weeks after hospitalization. Post-mortem analysis revealed large CD30- and CD45-positive lymphoma cells in an intravascular distribution in most of the organs studied. Histopathology of the spleen and bone marrow was significant for fulminant hemophagocytosis.

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References

  1. Ansell J, Bhawan J, Cohen S, et al (1982) Histiocytic lymphoma and malignant angioendotheliomatosis. One disease or two? Cancer 50:1506–1512

    CAS  PubMed  Google Scholar 

  2. Bogomolski-Yahalom V, Lossos IS, Okun E, et al (1998) Intravascular lymphomatosis—an indolent or aggressive entity? Leuk Lymphoma 29:585–593

    CAS  PubMed  Google Scholar 

  3. Brenner D, Buck M, Feitelberg S, et al (1990) Tumor necrosis factor-alpha inhibits albumin gene expression in a murine model of cachexia. J Clin Invest 85:248–255

    CAS  PubMed  Google Scholar 

  4. Dewald GW, Stupca P (2000) 154 chromosome anomalies in hematologic malignancies. Leuk Res 24:487–489

    Article  CAS  PubMed  Google Scholar 

  5. Glass J, Hochberg F, Miller D (1993) Intravascular lymphoma—a systemic disease with neurologic manifestations. Cancer 71:3156–3164

    CAS  PubMed  Google Scholar 

  6. Hennig B, Honchel R, Goldblum S, et al (1988) Tumor necrosis factor-mediated hypoalbuminemia in rabbits. J Nutr 118:1586–1590

    CAS  PubMed  Google Scholar 

  7. Ip M, Chan KW, Chan IK (1997) Systemic inflammatory response syndrome in intravascular lymphomatosis. Intensive Care Med 23:783–786

    Article  CAS  PubMed  Google Scholar 

  8. Koizumi M, Nishimura M, Yokota A, et al (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 

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

    CAS  PubMed  Google Scholar 

  10. 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 hemophagocytic syndrome. Br J Haematol 111:826–834

    Article  CAS  PubMed  Google Scholar 

  11. Pfleger L, Tappeiner J (1959) Zur Kenntnis der systemisierten Endotheliomatose der cutanen Blutgefasse. Hautarzt 10:359–363

    CAS  Google Scholar 

  12. Real E, Gomez A, Alcaraz M, et al (2000) Fulminant hemophagocytic syndrome as presenting feature of T-cell lymphoma and Epstein-Barr virus infection. Haematologica 85:439–440

    CAS  PubMed  Google Scholar 

  13. Shimazaki C, Inaba T, Nakagawa M (2000) B-cell lymphoma-associated hemophagocytic syndrome. Leuk Lymphoma 38:121–130

    CAS  PubMed  Google Scholar 

  14. Suzumiya J, Ohshima K, Kanda M, et al (1998) Intravascular large cell lymphoma associated with hypoalbuminemia. Leuk Lymphoma 32:179–182

    CAS  PubMed  Google Scholar 

  15. Yamaguchi M, Kimura M, Watanabe Y, et al (2001) Successful autologous peripheral blood stem cell transplantation for relapsed intravascular lymphomatosis. Bone Marrow Transplant 27:899–811

    Article  PubMed  Google Scholar 

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Correspondence to Niyati S. Bhagwati.

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Bhagwati, N.S., Oiseth, S.J., Abebe, L.S. et al. Intravascular lymphoma associated with hemophagocytic syndrome: a rare but aggressive clinical entity. Ann Hematol 83, 247–250 (2004). https://doi.org/10.1007/s00277-003-0757-3

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  • DOI: https://doi.org/10.1007/s00277-003-0757-3

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