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IgM Monoclonal Gammopathy of Undetermined Significance

MedGen UID:
647618
Concept ID:
C0563312
Neoplastic Process
Synonyms: IgM monoclonal gammopathy of uncertain significance; IgM monoclonal gammopathy of undetermined significance; Immunoglobulin M monoclonal gammopathy of uncertain significance; Immunoglobulin M monoclonal gammopathy of undetermined significance
SNOMED CT: Immunoglobulin M monoclonal gammopathy of uncertain significance (285426000); IgM monoclonal gammopathy of undetermined significance (788560004); Immunoglobulin M monoclonal gammopathy of undetermined significance (788560004); IgM monoclonal gammopathy of uncertain significance (285426000)

Definition

Monoclonal gammopathy of undetermined significance defined by a serum IgM paraprotein concentration less than 30g/L; bone marrow lymphoplasmacytic infiltration of less than 10%; and no evidence of anemia, constitutional symptoms, hyperviscocity, lymphadenopathy, hepatosplenomegaly, or other end-organ damage that can be attributed to the underlying lymphoproliferative disorder. It is a precursor condition that may progress to lymphoplasmacytic lymphoma/ Waldenstrom macroglobulinemia, other B-cell neoplasms, or primary amyloidosis. Progression to plasma cell myeloma occurs rarely, if at all. (WHO 2017) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIgM Monoclonal Gammopathy of Undetermined Significance

Professional guidelines

PubMed

Tomkins O, Leblond V, Lunn MP, Viala K, Weil DR, D'Sa S
Hematol Oncol Clin North Am 2023 Aug;37(4):761-776. doi: 10.1016/j.hoc.2023.04.007. PMID: 37385714
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
Gertz MA
Am J Hematol 2019 Feb;94(2):266-276. Epub 2018 Oct 17 doi: 10.1002/ajh.25292. PMID: 30328142

Recent clinical studies

Etiology

Tomkins O, Leblond V, Lunn MP, Viala K, Weil DR, D'Sa S
Hematol Oncol Clin North Am 2023 Aug;37(4):761-776. doi: 10.1016/j.hoc.2023.04.007. PMID: 37385714
Kaur J, Valisekka SS, Hameed M, Bandi PS, Varma S, Onwughalu CJ, Ibrahim H, Mongia H
Clin Lymphoma Myeloma Leuk 2023 May;23(5):e195-e212. Epub 2023 Feb 23 doi: 10.1016/j.clml.2023.02.004. PMID: 36966041
Alcoceba M, García-Álvarez M, Medina A, Maldonado R, González-Calle V, Chillón MC, Sarasquete ME, González M, García-Sanz R, Jiménez C
Int J Mol Sci 2022 May 16;23(10) doi: 10.3390/ijms23105570. PMID: 35628381Free PMC Article
Mailankody S, Landgren O
Best Pract Res Clin Haematol 2016 Jun;29(2):187-193. Epub 2016 Sep 4 doi: 10.1016/j.beha.2016.08.015. PMID: 27825465
Kyle RA, Dispenzieri A, Kumar S, Larson D, Therneau T, Rajkumar SV
Clin Lymphoma Myeloma Leuk 2011 Feb;11(1):74-6. doi: 10.3816/CLML.2011.n.011. PMID: 21454195

Diagnosis

Khwaja J, Vos JMI, Pluimers TE, Japzon N, Patel A, Salter S, Kwakernaak AJ, Gupta R, Rismani A, Kyriakou C, Wechalekar AD, D'Sa S
Br J Haematol 2024 Jan;204(1):177-185. Epub 2023 Sep 19 doi: 10.1111/bjh.19112. PMID: 37726004
Tomkins O, Leblond V, Lunn MP, Viala K, Weil DR, D'Sa S
Hematol Oncol Clin North Am 2023 Aug;37(4):761-776. doi: 10.1016/j.hoc.2023.04.007. PMID: 37385714
Bruehl FK, Mannion P, Barbato E, Nakashima MO, Cook JR
Haematologica 2023 Oct 1;108(10):2764-2773. doi: 10.3324/haematol.2022.282389. PMID: 37021542Free PMC Article
Kaur J, Valisekka SS, Hameed M, Bandi PS, Varma S, Onwughalu CJ, Ibrahim H, Mongia H
Clin Lymphoma Myeloma Leuk 2023 May;23(5):e195-e212. Epub 2023 Feb 23 doi: 10.1016/j.clml.2023.02.004. PMID: 36966041
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

Therapy

Castellani F, Visentin A, Schirinzi E, Salvalaggio A, Cacciavillani M, Candiotto C, Baratè C, Cellini A, Bertorelle R, Siciliano G, Trentin L, Briani C
Neurol Neuroimmunol Neuroinflamm 2023 Jul;10(4) Epub 2023 May 3 doi: 10.1212/NXI.0000000000200122. PMID: 37137530Free PMC Article
Nicol C, Pan-Petesch B, Ianotto JC
Haemophilia 2022 Nov;28(6):938-949. Epub 2022 Aug 25 doi: 10.1111/hae.14648. PMID: 36006003
Thomas A, Mailankody S, Korde N, Kristinsson SY, Turesson I, Landgren O
Blood 2012 Mar 22;119(12):2731-7. Epub 2012 Feb 6 doi: 10.1182/blood-2011-12-381426. PMID: 22310913Free PMC Article
Ramchandren S, Lewis RA
Curr Neurol Neurosci Rep 2012 Feb;12(1):102-10. doi: 10.1007/s11910-011-0237-4. PMID: 22090258
Matà S, Borsini W, Ambrosini S, Toscani L, Barilaro A, Piacentini S, Sorbi S, Lolli F
Eur J Neurol 2011 Aug;18(8):1067-73. Epub 2011 Jan 25 doi: 10.1111/j.1468-1331.2010.03345.x. PMID: 21261794

Prognosis

Khwaja J, Vos JMI, Pluimers TE, Japzon N, Patel A, Salter S, Kwakernaak AJ, Gupta R, Rismani A, Kyriakou C, Wechalekar AD, D'Sa S
Br J Haematol 2024 Jan;204(1):177-185. Epub 2023 Sep 19 doi: 10.1111/bjh.19112. PMID: 37726004
Moreno DF, López-Guerra M, Paz S, Oliver-Caldés A, Mena MP, Correa JG, Battram AM, Osuna M, Rivas-Delgado A, Rodríguez-Lobato LG, Cardús O, Tovar N, Cibeira MT, Jiménez-Segura R, Bladé J, Rosiñol L, Colomer D, Fernández de Larrea C
Br J Haematol 2023 Jan;200(2):187-196. Epub 2022 Oct 9 doi: 10.1111/bjh.18502. PMID: 36210485Free PMC Article
Alcoceba M, García-Álvarez M, Medina A, Maldonado R, González-Calle V, Chillón MC, Sarasquete ME, González M, García-Sanz R, Jiménez C
Int J Mol Sci 2022 May 16;23(10) doi: 10.3390/ijms23105570. PMID: 35628381Free PMC Article
van de Donk NW, Palumbo A, Johnsen HE, Engelhardt M, Gay F, Gregersen H, Hajek R, Kleber M, Ludwig H, Morgan G, Musto P, Plesner T, Sezer O, Terpos E, Waage A, Zweegman S, Einsele H, Sonneveld P, Lokhorst HM; European Myeloma Network
Haematologica 2014 Jun;99(6):984-96. Epub 2014 Mar 21 doi: 10.3324/haematol.2013.100552. PMID: 24658815Free PMC Article
Kyle RA, Benson J, Larson D, Therneau T, Dispenzieri A, Melton Iii LJ, Rajkumar SV
Clin Lymphoma Myeloma 2009 Mar;9(1):17-8. doi: 10.3816/CLM.2009.n.002. PMID: 19362962Free PMC Article

Clinical prediction guides

Khwaja J, Vos JMI, Pluimers TE, Japzon N, Patel A, Salter S, Kwakernaak AJ, Gupta R, Rismani A, Kyriakou C, Wechalekar AD, D'Sa S
Br J Haematol 2024 Jan;204(1):177-185. Epub 2023 Sep 19 doi: 10.1111/bjh.19112. PMID: 37726004
Castellani F, Visentin A, Schirinzi E, Salvalaggio A, Cacciavillani M, Candiotto C, Baratè C, Cellini A, Bertorelle R, Siciliano G, Trentin L, Briani C
Neurol Neuroimmunol Neuroinflamm 2023 Jul;10(4) Epub 2023 May 3 doi: 10.1212/NXI.0000000000200122. PMID: 37137530Free PMC Article
Bruehl FK, Mannion P, Barbato E, Nakashima MO, Cook JR
Haematologica 2023 Oct 1;108(10):2764-2773. doi: 10.3324/haematol.2022.282389. PMID: 37021542Free PMC Article
Mondello P, Paludo J, Novak JP, Wenzl K, Yang ZZ, Jalali S, Krull JE, Braggio E, Dasari S, Manske MK, Abeykoon JA, Sarangi V, Kapoor P, Paulus A, Reeder CB, Ailawadhi S, Chanan-Khan AA, Kyle RA, Gertz MA, Novak AJ, Ansell SM
Clin Cancer Res 2023 Mar 1;29(5):957-970. doi: 10.1158/1078-0432.CCR-22-2215. PMID: 36534518
McMaster ML
Hematol Oncol Clin North Am 2018 Oct;32(5):787-809. Epub 2018 Jul 26 doi: 10.1016/j.hoc.2018.05.006. PMID: 30190018

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