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1668 Part XI: Malignant Lymphoid Diseases Chapter 101: Marginal Zone B-cell Lymphomas 1669
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Pediatric NMZL is a different clinical entity that arises in adolescent 15. Dreyling M, Thieblemont C, Gallamini A, et al: ESMO consensus conferences:
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1
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tion of a large IgM paraprotein spike in the serum suggests LPL rather low-grade B-cell mucosa-associated lymphoid tissue lymphoma with prominent gastric
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tissue type treated with cladribine: 6 year follow-up of a phase II trial. Ann Oncol
No specific treatment consensus guidelines have been developed for 17:1722–1723, 2006.
NMZL, and patients are generally well managed according to treatment 23. Martinelli G, Laszlo D, Ferreri A, et al: Clinical activity of rituximab in gastric marginal
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In limited stage disease, surgery and radiotherapy are often appropriate, 24. Raderer M, Chott A, Drach J, et al: Chemotherapy for management of localised high
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concomitant HCV infection and no urgency for lymphoma treatment 25. Conconi A, Martinelli G, Lopez-Guillermo A, et al: Clinical activity of bortezomib in
relapsed/refractory MALT lymphomas: Results of a phase II study of the International
should receive antiviral treatment with interferon and ribavirin. High- Extranodal Lymphoma Study Group (IELSG). Ann Oncol 22:689–695, 2011.
47
dose therapy and autologous stem cell transplantation can be considered 26. Salar A, Domingo E, Canales M, et al: Bendamustine and rituximab as first line treat-
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COURSE AND PROGNOSIS 28. Zinzani PL, Pellegrini C, Broccoli A, et al: Fludarabine-mitoxantrone-rituximab regi-
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with an estimated 5-year event-free survival of nearly 30 percent, thus 29. Zucca E, Conconi A, Laszlo D, et al: Addition of rituximab to chlorambucil produces
indicating a high tendency of disease to relapse over time, albeit with superior event-free survival in the treatment of patients with extranodal marginal-
zone B-cell lymphoma: 5-year analysis of the IELSG-19 randomized study. J Clin Oncol
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