Abstract
Non-Hodgkin lymphomas (NHL) may be aggressive or indolent (“low-grade”). The paradox is that aggressive lymphomas are potentially curable, and patients who do not respond to initial therapy have a worse prognosis and short survival time. In contrast, the majority of patients with indolent lymphoma are not curable, respond to many different therapeutic interventions, and live a long period of time. Indolent follicular lymphomas arise from cells that populate lymph nodes and the bone marrow but may also involve extranodal sites. The World Health Organization (WHO) classification does not divide lymphomas by grade, and because they are not indolent, the preferred name used is “small B-cell lymphomas” in the most recent classification. The small B-cell lymphomas discussed in this section include follicular lymphoma (FL) (follicular lymphoma, in situ follicular lymphoma, duodenal-type FL, and predominantly diffuse follicular lymphoma with 1p36 deletion), nodal marginal zone lymphoma, splenic marginal zone lymphoma, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma, lymphoplasmacytic lymphoma/Waldenström macroglobulinemia, and primary cutaneous follicle center lymphoma. The updated and revised edition of the 2008 WHO classification was published in a paper in 2016 with a revised edition of the book released in 2017 (Swerdlow et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC, 2008; Swerdlow et al. Blood 127:2375–2390, 2016). The prognosis and treatment are dependent upon the histology, stage, amount or bulk of disease, symptoms, and other factors.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Suggested Reading
Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2008.
Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127:2375–90.
Al-Hamadani M, Habermann TM, Cerhan JR. Non-Hodgkin lymphoma subtype distribution, geodemographic patterns, and survival in the US: a longitudinal analysis of the National Cancer Data Base from 1998 to 2011. Am J Hematol. 2015;90:790–5.
Teras LR, DeSanto GE, Cerhan JR, et al. 2016 US lymphoid malignancy status in WHO subtypes. CA Cancer J Clin. 2016;66:443–59.
Linet MS, Vajdic CM, Morton LM, et al. Medical history, lifestyle, family history, and occupational risk factors for follicular lymphoma: the InterLymph non-Hodgkin Lymphoma Subtypes Project. J Natl Cancer Inst Monogr. 2014;48:26–40.
Habermann TM, Steensma DP. Lymphadenopathy. Mayo Clin Proc. 2000;75:723–32.
Brice P, Bastion Y, Lepage E, et al. Comparison in low-tumor-burden follicular lymphomas between and initial no treatment policy, prednimustine, and interferon alfa: a randomized study from the Groupe d’Edude des Lymphomes Folliculares-Groupe d’Etude des Lymphomes de l’Adulte. J Clin Oncol. 1997;15:1110–7.
Ardeshna KM, Qian W, Smith P, et al. Rituximab versus a watch-and-wait approach in patients with advanced-stage, asymptomatic, non-bulky follicular lymphoma: an open-label randomized phase 3 trial. Lancet Oncol. 2014;15:424–35.
Kahl BS, Hong F, Williams ME, et al. Rituximab extended schedule or re-treatment trial for low-tumor burden follicular lymphoma: Eastern Cooperative Oncology Group protocol E4402. J Clin Oncol. 2014;32:3096–102.
Wagner LI, Zhao F, Hong F, et al. Anxiety and health-related quality of life among patients with low-tumor burden non-Hodgkin lymphoma randomly assigned to two different rituximab dosing regimens: results from ECOG trial E4402 (RESORT). J Clin Oncol. 2015;33:740–8.
Friedberg JW, Taylor MD, Cerhan JR, et al. Follicular lymphoma in the United States: first report of the National LymphoCare Study. J Clin Oncol. 2009;27:1202–8.
Link BK, Maurer MJ, Nowakowski G, et al. Rates and outcomes of follicular lymphoma transformation in the immunochemotherapy era: a report from the University of Iowa/Mayo Clinic Specialized Program of Research Excellence Molecular Epidemiology Resource. J Clin Oncol. 2013;31:3272–8.
Maurer MJ, Bachy E, Ghesquiéres H, et al. Early event status informs subsequent outcome in newly diagnosed follicular lymphoma. Am J Hematol. 2016;91:1096–101.
Nooka AK, Nabhan C, Zhou X, et al. Examination of the follicular lymphoma international prognostic index (FLIPI) in the National LymphoCare study (NLCS): a prospective US patient cohort treated predominantly in community practices. Ann Oncol. 2013;24:441–8.
Villa D, Crump M, Panzarella T, et al. Autologous and allogeneic stem-cell transplantation for transformed follicular lymphoma: a report of the Canadian blood and marrow transplant group. J Clin Oncol. 2013;31:1164–71.
Al Khabori M, de Almeida JR, Guyatt GH, et al. Autologous stem cell transplantation in follicular lymphoma: a systemic review and meta-analysis. J Natl Cancer Inst. 2012;104:18–28.
van Besien K, Loberiza FR Jr, Bajorunaite R, et al. Comparison of autologous and allogeneic stem cell transplantation for follicular lymphoma. Blood. 2003;102:3521–9.
Salles GA, Morschhauser F, Solal-Celigny P, et al. Obinutuzumab (GA101) in patients with relapsed/refractory indolent non-Hodgkin lymphoma: results from the phase II GAUGUIN study. J Clin Oncol. 2013;31:2920–6.
Sehn LH, Chua N, Mayer J, et al. Obinutuzumab plus bendamustine versus bendamustine monotherapy in patients with rituximab-refractory indolent non-Hodgkin lymphoma (GADOLIN): a randomized, open-label, multicenter, phase 3 trial. Lancet Oncol. 2016;17:1081–8.
Witzig TE, Nowakowski GS, Habermann TM, et al. A comprehensive review of lenalidomide therapy for B-cell non-Hodgkin lymphoma. Ann Oncol. 2015;26:1667–77.
Witzig TE, Nowakowski GS, Habermann TM, et al. Long-term analysis of phase II studies of single-agent lenalidomide in relapsed/refractory mantle cell lymphoma. Am J Hematol Accepted July 11. 2017. https://doi.org/10.1002/ajh.24854.
Fowler N, Nastoupil L, de Vos S, et al. Ibrutinib plus rituximab in treatment-Naïve patients with follicular lymphoma: results from a multicenter, phase 2 study. Blood. 2015;126:470. Abstract 2015.
Fowler NH, Davis RE, Rawal S, et al. Safety and activity of lenalidomide and rituximab in untreated indolent lymphoma: an open-label, phase 2 trial. Lancet Oncol. 2014;15:1311–8.
Davids MS, Roberts AW, Seymour JF, et al. Phase 1 first-in-human study of venetoclax in patients with relapsed or refractory non-Hodgkin lymphoma. J Clin Oncol. 2017. https://doi.org/10.1200/JCO.2016.70.4320.
Lowry L, Smith P, Qian W, et al. Reduced dose radiotherapy for local control in non-Hodgkin lymphoma: a randomized phase III trial. Radiother Oncol. 2011;100:85–92.
Correia C, Schneider PA, Dai H, et al. BCL2 mutations are associated with increased risk of transformation and shortened survival in follicular lymphoma. Blood. 2015;125:658–67.
Zucca E, Copie-Bergman C, Ricardi U, et al. Gastric marginal zone lymphoma of MALT type: ESMO clinical practice guidelines for diagnosis, treatment, and follow-up. Ann Oncol. 2013;24(Supplement 6):vi144–8.
Zullo A, Hassan C, Cristofari F, et al. Effects of Helicobacter pylori eradication on early stage gastric mucosa-associated lymphoid tissue lymphoma. Clin Gastroenterol Hepatol. 2010;8:105–10.
Schechter NR, Portlock CS, Yahalom J, et al. Treatment of mucosa-associated lymphoid tissue lymphoma of the stomach with radiation alone. J Clin Oncol. 1998;16:1916–21.
Martinelli G, Laszlo D, Ferreri AJ, et al. Clinical activity of rituximab in gastric marginal zone non-Hodgkin lymphoma resistant to or not eligible for anti-Helicobacter pylori therapy. J Clin Oncol. 2005;23:1979–83.
Sammassimo S, Pruneri G, Andreola G, et al. A retrospective international study on primary extranodal marginal zone lymphoma of the lung (BALT lymphoma) on behalf of International Extranodal Lymphoma Study Group (IELSG). Hematol Oncol. 2016;34:177–83.
Jackson ME, Mian M, Kalpakais CH, et al. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue of the salivary glands: a multicenter, international experience of 248 patients (IELSG 41). Oncologist. 2015;20:1149–53.
Taner T, Nagorney DM, Tefferi A, et al. Splenectomy for massive splenomegaly. Ann Surg. 2013;258:1034–9.
Williams ME, Hong F, Gascoyne RD, et al. Rituximab extended schedule or retreatment trial for low tumour burden non-follicular indolent B-cell non-Hodgkin lymphomas: Eastern Cooperative Oncology Group Protocol E4402. Br J Haematol. 2016;173:867–75.
Kapoor P, Ansell SM, Fonseca R, et al. Diagnosis and management of Waldenström macroglobulinemia Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines 2016. JAMA Oncol. 2017. https://doi.org/10.1001/jamaoncol.2016.5763. Epub ahead of print.
Treon SP, Tripas CK, Meid K, et al. Ibrutinib in previously treated Waldenström’s Macroglobulinemia. N Engl J Med. 2015;372:1430–40.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2019 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Habermann, T.M. (2019). Indolent Lymphomas. In: Lazarus, H., Schmaier, A. (eds) Concise Guide to Hematology. Springer, Cham. https://doi.org/10.1007/978-3-319-97873-4_31
Download citation
DOI: https://doi.org/10.1007/978-3-319-97873-4_31
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-97872-7
Online ISBN: 978-3-319-97873-4
eBook Packages: MedicineMedicine (R0)