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IgG-associated immune thrombocytopenia in Waldenström macroglobulinemia

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Abstract

Waldenström macroglobulinemia (WM) is characterized by serum IgM monoclonal gammopathy, and only occasionally complicated by immune thrombocytopenia. Rarely, coexistence of non-IgM gammopathy in WM has been reported. Herein, we describe an 81-year-old case of WM with rapidly progressive immune thrombocytopenia accompanied by concurrent IgG gammopathy and elevation of platelet-associated IgG (PA-IgG). Immunostaining of the bone marrow specimen displayed not only IgM positive but also IgG positive plasmacytoid cells. Although dexamethasone therapy had no effect on thrombocytopenia, a modified RCD regimen (rituximab 375 mg/m2 i.v. day 1, cyclophosphamide 375 mg/m2 i.v. day 2 and dexamethasone 12 mg/body orally days 1–7) successfully normalized PA-IgG as well as IgG and IgM paraproteinemia and significantly increased platelet count. The current case suggests that the IgG gammopathy associated with WM is likely to be an etiology of immune thrombocytopenia and that tumor reduction, rather than immunosuppression, leads to an improvement of concurrent thrombocytopenia.

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References

  1. Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML, et al. Clinicopathological definition of Waldenstrom’s macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom’s Macroglobulinemia. Semin Oncol. 2003;30:110–5.

    Article  PubMed  Google Scholar 

  2. Dimopoulos MA, Gertz MA, Kastritis E, Garcia-Sanz R, Kimby EK, Leblond V, et al. Update on treatment recommendations from the Fourth International Workshop on Waldenstrom’s Macroglobulinemia. J Clin Oncol. 2009;27:120–6.

    Article  PubMed  Google Scholar 

  3. Dimopoulos MA, Panayiotidis P, Moulopoulos LA, Sfikakis P, Dalakas M. Waldenström’s macroglobulinemia: clinical features, complications, and management. J Clin Oncol. 2000;18:214–26.

    CAS  PubMed  Google Scholar 

  4. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: IARC Press; 2008. p. 194–5.

  5. Kyle RA, Robinson RA, Katzmann JA. The clinical aspects of biclonal gammopathies. Review of 57 cases. Am J Med. 1981;71:999–1008.

    Article  CAS  PubMed  Google Scholar 

  6. Weinstein S, Jain A, Bhagavan NV, Scottolini AG. Biclonal IgA and IgM gammopathy in lymphocytic lymphoma. Clin Chem. 1984;30:1710–2.

    CAS  PubMed  Google Scholar 

  7. Bonewald L, Virella G, Wang AC. Evidence for the biclonal nature of a Waldenstrom’s macroglobulinemia. Clin Chim Acta. 1985;146:53–63.

    Article  CAS  PubMed  Google Scholar 

  8. Schulz R, David D, Farkas DH, Crisan D. Molecular analysis in a patient with Waldenström’s macroglobulinemia reveals a rare case of biclonality. Mol Diagn. 1996;1:159–66.

    Article  PubMed  Google Scholar 

  9. Osterborg A, Mellstedt H. Monoclonal and biclonal immunoglobulin-producing disorders. Eur J Haematol Suppl. 1989;51:11–8.

    CAS  PubMed  Google Scholar 

  10. Fine JM, Gorin NC, Gendre JP, Petitpierre JC, Labro-Bryskier MT, Lambin P. Simultaneous occurrence of clinical manifestations of myeloma and Waldenström’s macroglobulinemia with monoclonal IgG Lambda and IgM Kappa in a single patient. Acta Med Scand. 1981;209:229–34.

    Article  CAS  PubMed  Google Scholar 

  11. Patel TC, Moore SB, Pineda AA, Witzig TE. Role of plasmapheresis in thrombocytopenic purpura associated with Waldenström’s macroglobulinemia. Mayo Clin Proc. 1996;71:597–600.

    Article  CAS  PubMed  Google Scholar 

  12. Kyrtsonis MC, Vassilakopoulos TP, Angelopoulou MK, Siakantaris P, Kontopidou FN, Dimopoulou MN, et al. Waldenström’s macroglobulinemia: clinical course and prognostic factors in 60 patients. Experience from a single hematology unit. Ann Hematol. 2001;80:722–7.

    Article  CAS  PubMed  Google Scholar 

  13. Owen RG, Lubenko A, Savage J, Parapia LA, Jack AS, Morgan GJ. Autoimmune thrombocytopenia in Waldenström’s macroglobulinemia. Am J Hematol. 2001;66:116–9.

    Article  CAS  PubMed  Google Scholar 

  14. Jønsson V, Kierkegaard A, Salling S, Molander S, Andersen LP, Christiansen M, Wiik A. Autoimmunity in Waldenström’s macroglobulinaemia. Leuk Lymphoma. 1999;34:373–9.

    PubMed  Google Scholar 

  15. Varticovski L, Pick AI, Schattner A, Shoenfeld Y. Anti-platelet and anti-DNA IgM in Waldenström’s macroglobulinemia and ITP. Am J Hematol. 1987;24:351–5.

    Article  CAS  PubMed  Google Scholar 

  16. Pawha J, Giuliani D, Morse BS. Platelet-associated IgM levels in thrombocytopenia. Vox Sang. 1983;45:97–103.

    Article  CAS  PubMed  Google Scholar 

  17. Lehman HA, Lehman LO, Rustagi PK, Rustgi RN, Plunkett RW, Farolino DL, et al. Complement-mediated autoimmune thrombocytopenia. Monoclonal IgM antiplatelet antibody associated with lymphoreticular malignant disease. N Engl J Med. 1987;316:194–8.

    Article  CAS  PubMed  Google Scholar 

  18. Yamamoto K, Nagata K, Hamaguchi H. Elevated platelet-associated IgG in immune thrombocytopenic purpura associated with Waldenström’s macroglobulinemia. Am J Hematol. 2002;69:85–6.

    Article  PubMed  Google Scholar 

  19. Kaufman M, Limaye SA, Driscoll N, Johnson C, Caramanica A, Lebowicz Y, et al. A combination of rituximab, cyclophosphamide and dexamethasone effectively treats immune cytopenias of chronic lymphocytic leukemia. Leuk Lymphoma. 2009;50:892–9.

    Article  CAS  PubMed  Google Scholar 

  20. Gupta N, Kavuru S, Patel D, Janson D, Driscoll N, Ahmed S, Rai KR. Rituximab-based chemotherapy for steroid-refractory autoimmune hemolytic anemia of chronic lymphocytic leukemia. Leukemia. 2002;16:2092–5.

    Article  CAS  PubMed  Google Scholar 

  21. Dimopoulos MA, Anagnostopoulos A, Kyrtsonis MC, Kyrtsonis MC, Zervas K, Tsatalas C, et al. Primary treatment of Waldenström macroglobulinemia with dexamethasone, rituximab, and cyclophosphamide. J Clin Oncol. 2007;25:3344–9.

    Article  CAS  PubMed  Google Scholar 

  22. Sahota SS, Garand R, Mahroof R, Smith A, Juge-Morineau N, Stevenson FK, Bataille R. V(H) gene analysis of IgM-secreting myeloma indicates an origin from a memory cell undergoing isotype switch events. Blood. 1999;94:1070–6.

    CAS  PubMed  Google Scholar 

  23. Zaja F, Baccarani M, Mazza P, Bocchia M, Gugliotta L, Zaccaria A, et al. Dexamethasone plus rituximab yields higher sustained response rates than dexamethasone monotherapy in adults with primary immune thrombocytopenia. Blood. 2010;115:2755–62.

    Article  CAS  PubMed  Google Scholar 

  24. Hauswirth AW, Skrabs C, Schützinger C, Raderer M, Chott A, Valent P, et al. Autoimmune thrombocytopenia in non-Hodgkin’s lymphomas. Haematologica. 2008;93:447–50.

    Article  PubMed  Google Scholar 

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Correspondence to Mineo Kurokawa.

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Nakazaki, K., Hangaishi, A., Nakamura, F. et al. IgG-associated immune thrombocytopenia in Waldenström macroglobulinemia. Int J Hematol 92, 360–363 (2010). https://doi.org/10.1007/s12185-010-0639-0

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  • DOI: https://doi.org/10.1007/s12185-010-0639-0

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