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Splenic marginal zone lymphoma presenting as myelofibrosis associated with bone marrow involvement of lymphoma cells which secrete a large amount of TGF-β

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Abstract

We report here on a patient with splenic marginal zone lymphoma presenting diffuse fibrosis of bone marrow and spleen. After splenectomy and chemotherapy, bone marrow biopsy demonstrated an improvement of fibrosis. Plasma concentration of transforming growth factor (TGF)-β was much higher in this patient than in those of age-matched non-Hodgkin’s lymphoma patients (n=5) at diagnosis, decreasing after resolution of myelofibrosis. Immunostaining with the TGF-β antibody revealed that the lymphoma cells in bone marrow and spleen were positive for TGF-β. TGF-β secreted by tumor cells was thought to stimulate the growth of fibroblasts and synthesize collagen in bone marrow and splenic fibroblasts, and play an important role in the development of marrow and splenic fibrosis in this patient. This is the first report of a patient with splenic marginal zone lymphoma presenting as myelofibrosis associated with bone marrow involvement of lymphoma cells which secrete a large amount of TGF-β.

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References

  1. Gralnick HR, Harbor J, Vogel C (1971) Myelofibrosis in chronic granulocytic leukemia. Blood 37:152–162

    CAS  PubMed  Google Scholar 

  2. San Miguel JF, Gonzalez M, Canizo MC, Ojeda E, Orfao A, Caballero MD, Moro MJ, Fisac P, Lopez Borrasca A (1988) Leukemias with megakaryoblastic involvement: clinical, hematologic, and immunologic characteristics. Blood 72:402–407

    PubMed  Google Scholar 

  3. Burthem J, Cawley JC (1994) The bone marrow fibrosis of hairy cell leukemia is caused by the synthesis and assembly of a fibronectin matrix by the hairy cells. Blood 83:497–504

    CAS  PubMed  Google Scholar 

  4. Burstein SA, Malpass TW, Yee E, Kadin M, Brigden M, Adamson JW, Harker LA (1984) Platelet factor-4 excretion in myeloproliferative disease: implications for the aetiology of myelofibrosis. Br J Haematol 57:383–392

    CAS  PubMed  Google Scholar 

  5. Terui T, Niitsu Y, Mahara K, Fujisaki Y, Urushizaki Y, Mogi Y, Kohgo Y, Watanabe N, Ogura M, Saito H (1990) The production of transforming growth factor-beta in acute megakaryocytic leukemia and its possible implication in myelofibrosis. Blood 75:1540–1548

    CAS  PubMed  Google Scholar 

  6. Dalley A, Smith JM, Reilly JT, Neil SM (1996) Investigation of calmodulin and basic fibroblast growth factor (bFGF) in idiopathic myelofibrosis: evidence for a role of extracellular calmodulin in fibroblast proliferation. Br J Haematol 93:856–862

    Article  CAS  PubMed  Google Scholar 

  7. Rameshwar P, Chang VT, Gascon P (1996) Implication of CD44 in adhesion-mediated overproduction of TGF-beta and IL-1 in monocytes from patients with bone marrow fibrosis. Br J Haematol 93:22–29

    Article  CAS  PubMed  Google Scholar 

  8. Martyre MC, Le Bousse-Kerdiles MC, Romquin N, Chevillard S, Praloran V, Demory JL, Dupriez B (1997) Elevated levels of basic fibroblast growth factor in megakaryocytes and platelets from patients with idiopathic myelofibrosis. Br J Haematol 97:441–448

    CAS  PubMed  Google Scholar 

  9. Sakamaki S, Hirayama Y, Ohi S, Kuga T, Kuroda H, Niitsu Y (1997) Retardation of platelet recovery after peripheral blood stem cell transplantation is due to the decrease of bone marrow stromal thrombopoietin (abstract). Blood 90 [Suppl 1]:141a

  10. Hamada T, Yonetani N, Ueda C, Maesako Y, Akasaka H, Akasaka T, Ohno H, Kawakami K, Amakawa R, Okuma M (1998) Expression of the PAX5/BSAP transcription factor in haematological tumour cells and further molecular characterization of the t(9;14)(p13;q32) translocation in B-cell non-Hodgkin’s lymphoma. Br J Haematol 102:691–700

    Article  CAS  PubMed  Google Scholar 

  11. Manoharan A, Horsley R, Pitney WR (1979) The reticulin content of bone marrow in acute leukaemia in adults. Br J Haematol 43:185–190

    CAS  PubMed  Google Scholar 

  12. Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC (1994) A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood 84:1361–1392

    CAS  PubMed  Google Scholar 

  13. Berger F, Felman P, Thieblemont C, Pradier T, Baseggio L, Bryon PA, Salles G, Callet-Bauchu E, Coiffier B (2000) Non-MALT marginal zone B-cell lymphomas: a description of clinical presentation and outcome in 124 patients. Blood 95:1950–1956

    CAS  PubMed  Google Scholar 

  14. Morrison AM, Jager U, Chott A, Schebesta M, Haas OA, Busslinger M (1998) Deregulated PAX-5 transcription from a translocated IgH promoter in marginal zone lymphoma. Blood 92:3865–3878

    CAS  PubMed  Google Scholar 

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Acknowledgements

The authors thank Mr. Kevin Litton (Bachelor of Arts in English) for editorial assistance.

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Correspondence to T. Matsunaga.

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Matsunaga, T., Takemoto, N., Miyajima, N. et al. Splenic marginal zone lymphoma presenting as myelofibrosis associated with bone marrow involvement of lymphoma cells which secrete a large amount of TGF-β. Ann Hematol 83, 322–325 (2004). https://doi.org/10.1007/s00277-003-0806-y

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  • DOI: https://doi.org/10.1007/s00277-003-0806-y

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