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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

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
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

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
Managing Waldenström's macroglobulinemia with BTK inhibitors.
Buske C, Jurczak W, Salem JE, Dimopoulos MA
Leukemia 2023 Jan;37(1):35-46. Epub 2022 Nov 19 doi: 10.1038/s41375-022-01732-9. PMID: 36402930Free 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

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
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
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
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
Managing Waldenström's macroglobulinemia with BTK inhibitors.
Buske C, Jurczak W, Salem JE, Dimopoulos MA
Leukemia 2023 Jan;37(1):35-46. Epub 2022 Nov 19 doi: 10.1038/s41375-022-01732-9. PMID: 36402930Free 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

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.
Baďurová K, Gregorová J, Vlachová M, Krejčí M, Ševčíková S
Klin Onkol 2021 Fall;34(6):428-433. doi: 10.48095/ccko2021428. PMID: 34911327
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

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
A revised international prognostic score system for Waldenström's macroglobulinemia.
Kastritis E, Morel P, Duhamel A, Gavriatopoulou M, Kyrtsonis MC, Durot E, Symeonidis A, Laribi K, Hatjiharissi E, Ysebaert L, Vassou A, Giannakoulas N, Merlini G, Repousis P, Varettoni M, Michalis E, Hivert B, Michail M, Katodritou E, Terpos E, Leblond V, Dimopoulos MA
Leukemia 2019 Nov;33(11):2654-2661. Epub 2019 May 22 doi: 10.1038/s41375-019-0431-y. PMID: 31118465
Waldenström macroglobulinemia.
Vijay A, Gertz MA
Blood 2007 Jun 15;109(12):5096-103. Epub 2007 Feb 15 doi: 10.1182/blood-2006-11-055012. PMID: 17303694

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
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
Systematic review of the recent evidence for the efficacy and safety of chlorambucil in the treatment of B-cell malignancies.
Lepretre S, Dartigeas C, Feugier P, Marty M, Salles G
Leuk Lymphoma 2016;57(4):852-65. Epub 2015 Oct 8 doi: 10.3109/10428194.2015.1085528. PMID: 26308278

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