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Waldenstrom macroglobulinemia

MedGen UID:
6174
•Concept ID:
C0024419
•
Neoplastic Process
Synonyms: Lymphoma, Lymphocytic, Plasmacytoid; Lymphoma, Lymphoplasmacytoid; Lymphomas, Lymphoplasmacytoid; Lymphoplasmacytoid Lymphoma; Lymphoplasmacytoid Lymphomas; Macroglobulinaemia, Waldenstrom's; Macroglobulinemia; Macroglobulinemia, Primary; Macroglobulinemia, Waldenstrom; Macroglobulinemia, Waldenstrom's; Primary Macroglobulinemia; Waldenstrom Macroglobulinaemia; Waldenstrom Macroglobulinemia; Waldenstrom's Macroglobulinaemia; Waldenstrom's Macroglobulinemia; Waldenstroms Macroglobulinaemia; Waldenstroms Macroglobulinemia
SNOMED CT: Waldenström macroglobulinemia (190818004); Waldenstrom macroglobulinemia (35562000); Macroglobulinemia (190817009); Waldenstrom's macroglobulinemia (190818004); Waldenstrom's macroglobulinemia (35562000); Primary macroglobulinemia (35562000)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Non-Mendelian inheritance (Orphanet)
 
Related gene: MYD88
 
Monarch Initiative: MONDO:0100280
OMIM® Phenotypic series: PS153600
Orphanet: ORPHA33226

Definition

Waldenström macroglobulinemia (WM) is an indolent B-cell lymphoproliferative disorder characterized by the accumulation of monoclonal cells in the bone marrow and peripheral lymphoid tissues, and associated with the production of serum immunoglobulin M (IgM) monoclonal protein. [from ORDO]

Professional guidelines

PubMed

IgM monoclonal gammopathies of clinical significance: diagnosis and management.
Khwaja J, D'Sa S, Minnema MC, Kersten MJ, Wechalekar A, Vos JM
Haematologica 2022 Sep 1;107(9):2037-2050. doi: 10.3324/haematol.2022.280953. PMID: 35770530Free PMC Article
Waldenström macroglobulinemia: 2021 update on diagnosis, risk stratification, and management.
Gertz MA
Am J Hematol 2021 Feb 1;96(2):258-269. doi: 10.1002/ajh.26082. PMID: 33368476
Oncologic Emergencies: Recognition and Initial Management.
Higdon ML, Atkinson CJ, Lawrence KV
Am Fam Physician 2018 Jun 1;97(11):741-748. PMID: 30215936

Recent clinical studies

Etiology

Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management.
Gertz MA
Am J Hematol 2023 Feb;98(2):348-358. Epub 2023 Jan 1 doi: 10.1002/ajh.26796. PMID: 36588395Free PMC Article
Monoclonal Gammopathy of Undetermined Significance.
Gonsalves WI, Rajkumar SV
Ann Intern Med 2022 Dec;175(12):ITC177-ITC192. Epub 2022 Dec 13 doi: 10.7326/AITC202212200. PMID: 36508741
Waldenstrom Macroglobulinemia: Tailoring Therapy for the Individual.
Gertz MA
J Clin Oncol 2022 Aug 10;40(23):2600-2608. Epub 2022 Jun 14 doi: 10.1200/JCO.22.00495. PMID: 35700418Free PMC Article
Waldenström macroglobulinemia: 2021 update on diagnosis, risk stratification, and management.
Gertz MA
Am J Hematol 2021 Feb 1;96(2):258-269. doi: 10.1002/ajh.26082. PMID: 33368476
A randomized phase 3 trial of zanubrutinib vs ibrutinib in symptomatic Waldenström macroglobulinemia: the ASPEN study.
Tam CS, Opat S, D'Sa S, Jurczak W, Lee HP, Cull G, Owen RG, Marlton P, Wahlin BE, Sanz RG, McCarthy H, Mulligan S, Tedeschi A, Castillo JJ, Czyz J, Fernández de Larrea C, Belada D, Libby E, Matous JV, Motta M, Siddiqi T, Tani M, Trneny M, Minnema MC, Buske C, Leblond V, Trotman J, Chan WY, Schneider J, Ro S, Cohen A, Huang J, Dimopoulos M
Blood 2020 Oct 29;136(18):2038-2050. doi: 10.1182/blood.2020006844. PMID: 32731259Free PMC Article

Diagnosis

Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management.
Gertz MA
Am J Hematol 2023 Feb;98(2):348-358. Epub 2023 Jan 1 doi: 10.1002/ajh.26796. PMID: 36588395Free PMC Article
Monoclonal Gammopathy of Undetermined Significance.
Gonsalves WI, Rajkumar SV
Ann Intern Med 2022 Dec;175(12):ITC177-ITC192. Epub 2022 Dec 13 doi: 10.7326/AITC202212200. PMID: 36508741
When a Monoclonal Gammopathy Is Not Multiple Myeloma.
Derman B, Castillo JJ, Sarosiek S, Beksac M
Am Soc Clin Oncol Educ Book 2022 Apr;42:1-10. doi: 10.1200/EDBK_349643. PMID: 35394823
Waldenström macroglobulinemia: 2021 update on diagnosis, risk stratification, and management.
Gertz MA
Am J Hematol 2021 Feb 1;96(2):258-269. doi: 10.1002/ajh.26082. PMID: 33368476
How I treat Waldenström macroglobulinemia.
Dimopoulos MA, Kastritis E
Blood 2019 Dec 5;134(23):2022-2035. doi: 10.1182/blood.2019000725. PMID: 31527073

Therapy

Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management.
Gertz MA
Am J Hematol 2023 Feb;98(2):348-358. Epub 2023 Jan 1 doi: 10.1002/ajh.26796. PMID: 36588395Free PMC Article
Current approach to Waldenström Macroglobulinemia.
Ravi G, Kapoor P
Cancer Treat Res Commun 2022;31:100527. Epub 2022 Feb 2 doi: 10.1016/j.ctarc.2022.100527. PMID: 35149375
A randomized phase 3 trial of zanubrutinib vs ibrutinib in symptomatic Waldenström macroglobulinemia: the ASPEN study.
Tam CS, Opat S, D'Sa S, Jurczak W, Lee HP, Cull G, Owen RG, Marlton P, Wahlin BE, Sanz RG, McCarthy H, Mulligan S, Tedeschi A, Castillo JJ, Czyz J, Fernández de Larrea C, Belada D, Libby E, Matous JV, Motta M, Siddiqi T, Tani M, Trneny M, Minnema MC, Buske C, Leblond V, Trotman J, Chan WY, Schneider J, Ro S, Cohen A, Huang J, Dimopoulos M
Blood 2020 Oct 29;136(18):2038-2050. doi: 10.1182/blood.2020006844. PMID: 32731259Free PMC Article
How I treat Waldenström macroglobulinemia.
Dimopoulos MA, Kastritis E
Blood 2019 Dec 5;134(23):2022-2035. doi: 10.1182/blood.2019000725. PMID: 31527073
Lymphoplasmacytic Lymphoma and Marginal Zone Lymphoma.
Juárez-Salcedo LM, Castillo JJ
Hematol Oncol Clin North Am 2019 Aug;33(4):639-656. Epub 2019 Apr 30 doi: 10.1016/j.hoc.2019.03.004. PMID: 31229160

Prognosis

Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management.
Gertz MA
Am J Hematol 2023 Feb;98(2):348-358. Epub 2023 Jan 1 doi: 10.1002/ajh.26796. PMID: 36588395Free PMC Article
Current approach to Waldenström Macroglobulinemia.
Ravi G, Kapoor P
Cancer Treat Res Commun 2022;31:100527. Epub 2022 Feb 2 doi: 10.1016/j.ctarc.2022.100527. PMID: 35149375
Waldenström macroglobulinemia: 2021 update on diagnosis, risk stratification, and management.
Gertz MA
Am J Hematol 2021 Feb 1;96(2):258-269. doi: 10.1002/ajh.26082. PMID: 33368476
A randomized phase 3 trial of zanubrutinib vs ibrutinib in symptomatic Waldenström macroglobulinemia: the ASPEN study.
Tam CS, Opat S, D'Sa S, Jurczak W, Lee HP, Cull G, Owen RG, Marlton P, Wahlin BE, Sanz RG, McCarthy H, Mulligan S, Tedeschi A, Castillo JJ, Czyz J, Fernández de Larrea C, Belada D, Libby E, Matous JV, Motta M, Siddiqi T, Tani M, Trneny M, Minnema MC, Buske C, Leblond V, Trotman J, Chan WY, Schneider J, Ro S, Cohen A, Huang J, Dimopoulos M
Blood 2020 Oct 29;136(18):2038-2050. doi: 10.1182/blood.2020006844. PMID: 32731259Free PMC Article
Lymphoplasmacytic Lymphoma and Marginal Zone Lymphoma.
Juárez-Salcedo LM, Castillo JJ
Hematol Oncol Clin North Am 2019 Aug;33(4):639-656. Epub 2019 Apr 30 doi: 10.1016/j.hoc.2019.03.004. PMID: 31229160

Clinical prediction guides

Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management.
Gertz MA
Am J Hematol 2023 Feb;98(2):348-358. Epub 2023 Jan 1 doi: 10.1002/ajh.26796. PMID: 36588395Free PMC Article
Ibrutinib Plus Rituximab Versus Placebo Plus Rituximab for Waldenström's Macroglobulinemia: Final Analysis From the Randomized Phase III iNNOVATE Study.
Buske C, Tedeschi A, Trotman J, García-Sanz R, MacDonald D, Leblond V, Mahe B, Herbaux C, Matous JV, Tam CS, Heffner LT, Varettoni M, Palomba ML, Shustik C, Kastritis E, Treon SP, Ping J, Hauns B, Arango-Hisijara I, Dimopoulos MA
J Clin Oncol 2022 Jan 1;40(1):52-62. Epub 2021 Oct 4 doi: 10.1200/JCO.21.00838. PMID: 34606378Free PMC Article
Waldenström macroglobulinemia: 2021 update on diagnosis, risk stratification, and management.
Gertz MA
Am J Hematol 2021 Feb 1;96(2):258-269. doi: 10.1002/ajh.26082. PMID: 33368476
Lymphoplasmacytic Lymphoma and Marginal Zone Lymphoma.
Juárez-Salcedo LM, Castillo JJ
Hematol Oncol Clin North Am 2019 Aug;33(4):639-656. Epub 2019 Apr 30 doi: 10.1016/j.hoc.2019.03.004. PMID: 31229160
MYD88 L265P somatic mutation in Waldenström's macroglobulinemia.
Treon SP, Xu L, Yang G, Zhou Y, Liu X, Cao Y, Sheehy P, Manning RJ, Patterson CJ, Tripsas C, Arcaini L, Pinkus GS, Rodig SJ, Sohani AR, Harris NL, Laramie JM, Skifter DA, Lincoln SE, Hunter ZR
N Engl J Med 2012 Aug 30;367(9):826-33. doi: 10.1056/NEJMoa1200710. PMID: 22931316

Recent systematic reviews

Efficacy and safety of front-line treatment regimens for Waldenstrom macroglobulinaemia: a systematic review and meta-analysis.
Chan WL, Chong VCL, Wee IJY, Poon LM, Chan EHL, Lee J, Chee YL, Jeyasekharan AD, Chng WJ, Samuel M, de Mel S
Blood Cancer J 2023 Sep 7;13(1):140. doi: 10.1038/s41408-023-00916-5. PMID: 37679351Free PMC Article
First- versus second-generation Bruton tyrosine kinase inhibitors in Waldenström's Macroglobulinemia: A systematic review and meta-analysis.
Abushukair H, Syaj S, Ababneh O, Qarqash A, Schinke C, Thanendrarajan S, Zangari M, van Rhee F, Al Hadidi S
Am J Hematol 2022 Jul;97(7):942-950. Epub 2022 Apr 8 doi: 10.1002/ajh.26552. PMID: 35358350
Priapism in Lymphoproliferative Disorders: A Systematic Review.
Ali EA, Sardar S, Yassin MA
Hematol Oncol Stem Cell Ther 2022 Dec 23;15(4):176-182. doi: 10.1016/j.hemonc.2021.05.003. PMID: 34157311
Efficacy of Autologous and Allogeneic Hematopoietic Cell Transplantation in Waldenström Macroglobulinemia: A Systematic Review and Meta-analysis.
Parrondo RD, Reljic T, Iqbal M, Ayala E, Tun HW, Kharfan-Dabaja MA, Kumar A, Murthy HS
Clin Lymphoma Myeloma Leuk 2020 Oct;20(10):e694-e711. Epub 2020 Jun 2 doi: 10.1016/j.clml.2020.05.021. PMID: 32616401
CXCR4 mutations affect presentation and outcomes in patients with Waldenström macroglobulinemia: A systematic review.
Castillo JJ, Moreno DF, Arbelaez MI, Hunter ZR, Treon SP
Expert Rev Hematol 2019 Oct;12(10):873-881. Epub 2019 Jul 30 doi: 10.1080/17474086.2019.1649132. PMID: 31343930

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