The interpretation of version 1. 2018 of the NCCN Guidelines for Waldenström’s macroglobulinemia/lymphoplasmacytic lymphoma_West China Medical Journal

Authors：

 NIU Ting

Department of Hematology/Research Laboratory of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

NIU Ting, Email: tingniu@sina.com

Keywords：

Waldenström’s macroglobulinemia; Lymphoplasmacytic lymphoma; Diagnosis; Therapy

DOI：

10.7507/1002-0179.201804003

Video：

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Waldenström’s macroglobulinemia (WM)/lymphoplasmacytic lymphoma (LPL) is a rare mature B-cell neoplasm. WM is characterized by the presence of a lymphoplasmacytic infiltrate in the bone marrow and high serum levels of monoclonal immunoglobulin M protein. With a deeper understanding of molecular mechanisms of the disease, the update of diagnostic approaches and the introduction of novel therapies, the management of WM/LPL has rapidly evolved over the past few years. On March 7th, 2018, National Comprehensive Cancer Network (NCCN) updated the guideline for WM/LPL. This study mainly interpreted the corresponding diagnosis and treatment of WM/LPL in NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®): Waldenström’s Macroglobulinemia/Lymphoplasmacytic Lymphoma (Version 1.2018).

Citation： NIU Ting. The interpretation of version 1. 2018 of the NCCN Guidelines for Waldenström’s macroglobulinemia/lymphoplasmacytic lymphoma. West China Medical Journal, 2018, 33(4): 393-397. doi: 10.7507/1002-0179.201804003 Copy

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2.	Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. WHO classification of tumours, Revised 4th edition, Volume 2. Geneva: WHO, 2017.
3.	Gertz MA. Waldenström macroglobulinemia: 2012 update on diagnosis, risk stratification, and management. Am J Hematol, 2012, 87(5): 503-510.
4.	Treon SP, Xu L, Zhou Y, et al. Whole genome sequencing reveals a widely expressed mutation (MYD88 L265P) with oncogenic activity in Waldenström’s macroglobulinemia. Blood, 2011, 118: Abstract 300.
5.	Treon SP, Xu L, Yang G, et al.MYD88 L265P somatic mutation in Waldenström’s macroglobulinemia. N Engl J Med, 2012, 367(9): 826-833.
6.	Jiménez C, Sebastián E, Chillón MC, et al. MYD88 L265P is a marker highly characteristic of, but not restricted to, Waldenström’s macroglobulinemia. Leukemia, 2013, 27(8): 1722-1728.
7.	Cao Y, Hunter ZR, Liu X, et al. The WHIM-like CXCR4 (S338X) somatic mutation activates AKT and ERK, and promotes resistance to ibrutinib and other agents used in the treatment of Waldenström’s macroglobulinemia. Leukemia, 2015, 29(1): 169-176.
8.	Kastritis E, Kyrtsonis MC, Hadjiharissi E, et al. Validation of the international prognostic scoring system (IPSS) for Waldenström’s macroglobulinemia (WM) and the importance of serum lactate dehydrogenase (LDH). Leuk Res, 2010, 34(10): 1340-1343.
9.	Rummel MJ, Niederle N, Maschmeyer G, et al. Bendamustine plus rituximab versus CHOP plus rituximab as first-line treatment for patients with indolent and mantle-cell lymphomas: an open-label, multicentre, randomised, phase 3 non-inferiority trial. Lancet, 2013, 381(9873): 1203-1210.
10.	Treon SP, Ioakimidis L, Soumerai JD, et al. Primary therapy of Waldenström macroglobulinemia with bortezomib, dexamethasone, and rituximab: WMCTG clinical trial 05-180. J Clin Oncol, 2009, 27(23): 3830-3835.
11.	Dimopoulos MA, Anagnostopoulos A, Kyrtsonis MC, et al. Primary treatment of Waldenström macroglobulinemia with dexamethasone, rituximab, and cyclophosphamide. J Clin Oncol, 2007, 25(22): 3344-3349.
12.	Treon SP, Hanzis C, Manning RJ, et al. Maintenance rituximab is associated with improved clinical outcome in rituximab naive patients with Waldenström’s macroglobulinaemia who respond to a rituximab-containing regimen. Br J Haematol, 2011, 154(3): 357-362.
13.	Treon SP, Tripsas CK, Meid K, et al. Ibrutinib in previously treated Waldenström’s macroglobulinemia. N Engl J Med, 2015, 372(15): 1430-1440.

1. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood, 2016, 127(20): 2375-2390.
2. Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. WHO classification of tumours, Revised 4th edition, Volume 2. Geneva: WHO, 2017.
3. Gertz MA. Waldenström macroglobulinemia: 2012 update on diagnosis, risk stratification, and management. Am J Hematol, 2012, 87(5): 503-510.
4. Treon SP, Xu L, Zhou Y, et al. Whole genome sequencing reveals a widely expressed mutation (MYD88 L265P) with oncogenic activity in Waldenström’s macroglobulinemia. Blood, 2011, 118: Abstract 300.
5. Treon SP, Xu L, Yang G, et al.MYD88 L265P somatic mutation in Waldenström’s macroglobulinemia. N Engl J Med, 2012, 367(9): 826-833.
6. Jiménez C, Sebastián E, Chillón MC, et al. MYD88 L265P is a marker highly characteristic of, but not restricted to, Waldenström’s macroglobulinemia. Leukemia, 2013, 27(8): 1722-1728.
7. Cao Y, Hunter ZR, Liu X, et al. The WHIM-like CXCR4 (S338X) somatic mutation activates AKT and ERK, and promotes resistance to ibrutinib and other agents used in the treatment of Waldenström’s macroglobulinemia. Leukemia, 2015, 29(1): 169-176.
8. Kastritis E, Kyrtsonis MC, Hadjiharissi E, et al. Validation of the international prognostic scoring system (IPSS) for Waldenström’s macroglobulinemia (WM) and the importance of serum lactate dehydrogenase (LDH). Leuk Res, 2010, 34(10): 1340-1343.
9. Rummel MJ, Niederle N, Maschmeyer G, et al. Bendamustine plus rituximab versus CHOP plus rituximab as first-line treatment for patients with indolent and mantle-cell lymphomas: an open-label, multicentre, randomised, phase 3 non-inferiority trial. Lancet, 2013, 381(9873): 1203-1210.
10. Treon SP, Ioakimidis L, Soumerai JD, et al. Primary therapy of Waldenström macroglobulinemia with bortezomib, dexamethasone, and rituximab: WMCTG clinical trial 05-180. J Clin Oncol, 2009, 27(23): 3830-3835.
11. Dimopoulos MA, Anagnostopoulos A, Kyrtsonis MC, et al. Primary treatment of Waldenström macroglobulinemia with dexamethasone, rituximab, and cyclophosphamide. J Clin Oncol, 2007, 25(22): 3344-3349.
12. Treon SP, Hanzis C, Manning RJ, et al. Maintenance rituximab is associated with improved clinical outcome in rituximab naive patients with Waldenström’s macroglobulinaemia who respond to a rituximab-containing regimen. Br J Haematol, 2011, 154(3): 357-362.
13. Treon SP, Tripsas CK, Meid K, et al. Ibrutinib in previously treated Waldenström’s macroglobulinemia. N Engl J Med, 2015, 372(15): 1430-1440.

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The interpretation of version 1. 2018 of the NCCN Guidelines for Waldenström’s macroglobulinemia/lymphoplasmacytic lymphoma

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