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IgM heavy chain paraproteinemia

MedGen UID:
1707667
•Concept ID:
C5209278
•
Finding
HPO: HP:0020196

Definition

An abnormal IgM heavy chain in the circulation and typically produced by a clonal population of B-cell derived plasma cells. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIgM heavy chain paraproteinemia

Professional guidelines

PubMed

Benefits of new immunoglobulin-derived biomarkers for the diagnosis and follow-up of patients with dysglobulinemia.
Ermak N, Nguyen-Khoa T, Alyanakian MA
Ann Biol Clin (Paris) 2016 Oct 1;74(5):597-605. doi: 10.1684/abc.2016.1180. PMID: 27707674
The clinical relevance and management of monoclonal gammopathy of undetermined significance and related disorders: recommendations from the European Myeloma Network.
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
Analysis of surface and cytoplasmic immunoglobulin light/heavy chains by flow cytometry using a lysed-whole-blood technique: Implications for the differential diagnosis of B-cell malignancies.
Babusíková O, Stevulová L
Neoplasma 2004;51(6):422-30. PMID: 15640950

Recent clinical studies

Etiology

Recovery of uninvolved heavy/light chain pair immunoparesis in newly diagnosed transplant-eligible myeloma patients complements the prognostic value of minimal residual disease detection.
Lakhwani S, Rosiñol L, Puig N, Pico-Picos MA, Medina-González L, Martínez-López J, Paiva B, Cedena MT, Oriol A, Ríos-Tamayo R, Blanchard MJ, Jarque I, Bargay J, Moraleda JM, Carrillo-Cruz E, Sureda A, Krsnik I, González E, Casado LF, Martí JM, Encinas C, De Arriba F, Palomera L, Sampol A, González-Montes Y, Motlló C, De La Cruz J, Alonso R, Mateos MV, Bladé J, Lahuerta JJ, San-Miguel J, Hernández MT
Haematologica 2024 Jun 1;109(6):1909-1917. doi: 10.3324/haematol.2023.284154. PMID: 38031761Free PMC Article
Benefits of new immunoglobulin-derived biomarkers for the diagnosis and follow-up of patients with dysglobulinemia.
Ermak N, Nguyen-Khoa T, Alyanakian MA
Ann Biol Clin (Paris) 2016 Oct 1;74(5):597-605. doi: 10.1684/abc.2016.1180. PMID: 27707674
T- and B-cell immune reconstitution and clinical outcome in patients with multiple myeloma receiving T-cell-depleted, reduced-intensity allogeneic stem cell transplantation with an alemtuzumab-containing conditioning regimen followed by escalated donor lymphocyte infusions.
D'Sa S, Peggs K, Pizzey A, Verfuerth S, Thuraisundaram D, Watts M, White H, Hale G, Waldmann H, Goldstone A, Mackinnon S, Yong K
Br J Haematol 2003 Oct;123(2):309-22. doi: 10.1046/j.1365-2141.2003.04612.x. PMID: 14531914
Waldenström's macroglobulinaemia.
Johnson SA, Oscier DG, Leblond V
Blood Rev 2002 Sep;16(3):175-84. doi: 10.1016/s0268-960x(02)00016-4. PMID: 12163003
Heavy chain disease.
Witzig TE, Wahner-Roedler DL
Curr Treat Options Oncol 2002 Jun;3(3):247-54. doi: 10.1007/s11864-002-0014-3. PMID: 12057070

Diagnosis

Top 10 Clinical Pearls in Paraproteinemic Neuropathies.
Becker B, Stino A
Semin Neurol 2025 Feb;45(1):99-111. Epub 2024 Oct 17 doi: 10.1055/s-0044-1791769. PMID: 39419069
Recovery of uninvolved heavy/light chain pair immunoparesis in newly diagnosed transplant-eligible myeloma patients complements the prognostic value of minimal residual disease detection.
Lakhwani S, Rosiñol L, Puig N, Pico-Picos MA, Medina-González L, Martínez-López J, Paiva B, Cedena MT, Oriol A, Ríos-Tamayo R, Blanchard MJ, Jarque I, Bargay J, Moraleda JM, Carrillo-Cruz E, Sureda A, Krsnik I, González E, Casado LF, Martí JM, Encinas C, De Arriba F, Palomera L, Sampol A, González-Montes Y, Motlló C, De La Cruz J, Alonso R, Mateos MV, Bladé J, Lahuerta JJ, San-Miguel J, Hernández MT
Haematologica 2024 Jun 1;109(6):1909-1917. doi: 10.3324/haematol.2023.284154. PMID: 38031761Free PMC Article
Benefits of new immunoglobulin-derived biomarkers for the diagnosis and follow-up of patients with dysglobulinemia.
Ermak N, Nguyen-Khoa T, Alyanakian MA
Ann Biol Clin (Paris) 2016 Oct 1;74(5):597-605. doi: 10.1684/abc.2016.1180. PMID: 27707674
Waldenström's macroglobulinaemia.
Johnson SA, Oscier DG, Leblond V
Blood Rev 2002 Sep;16(3):175-84. doi: 10.1016/s0268-960x(02)00016-4. PMID: 12163003
Heavy chain disease.
Witzig TE, Wahner-Roedler DL
Curr Treat Options Oncol 2002 Jun;3(3):247-54. doi: 10.1007/s11864-002-0014-3. PMID: 12057070

Therapy

Recovery of uninvolved heavy/light chain pair immunoparesis in newly diagnosed transplant-eligible myeloma patients complements the prognostic value of minimal residual disease detection.
Lakhwani S, Rosiñol L, Puig N, Pico-Picos MA, Medina-González L, Martínez-López J, Paiva B, Cedena MT, Oriol A, Ríos-Tamayo R, Blanchard MJ, Jarque I, Bargay J, Moraleda JM, Carrillo-Cruz E, Sureda A, Krsnik I, González E, Casado LF, Martí JM, Encinas C, De Arriba F, Palomera L, Sampol A, González-Montes Y, Motlló C, De La Cruz J, Alonso R, Mateos MV, Bladé J, Lahuerta JJ, San-Miguel J, Hernández MT
Haematologica 2024 Jun 1;109(6):1909-1917. doi: 10.3324/haematol.2023.284154. PMID: 38031761Free PMC Article
Systemic Amyloidosis due to Low-Grade Lymphoma.
Wechalekar AD, Chakraborty R, Lentzsch S
Hematol Oncol Clin North Am 2020 Dec;34(6):1027-1039. Epub 2020 Oct 1 doi: 10.1016/j.hoc.2020.08.016. PMID: 33099421
Familial predisposition to monoclonal gammopathies: deviations in B-cell biology.
Ögmundsdóttir HM, Valgeirsdóttir S, Schiffhauer HR, Óskarsdóttir LB, Steingrímsdóttir H, Haraldsdóttir V
Clin Lymphoma Myeloma Leuk 2013 Apr;13(2):191-3. Epub 2013 Mar 13 doi: 10.1016/j.clml.2013.02.017. PMID: 23490993
T- and B-cell immune reconstitution and clinical outcome in patients with multiple myeloma receiving T-cell-depleted, reduced-intensity allogeneic stem cell transplantation with an alemtuzumab-containing conditioning regimen followed by escalated donor lymphocyte infusions.
D'Sa S, Peggs K, Pizzey A, Verfuerth S, Thuraisundaram D, Watts M, White H, Hale G, Waldmann H, Goldstone A, Mackinnon S, Yong K
Br J Haematol 2003 Oct;123(2):309-22. doi: 10.1046/j.1365-2141.2003.04612.x. PMID: 14531914
Waldenström's macroglobulinaemia.
Johnson SA, Oscier DG, Leblond V
Blood Rev 2002 Sep;16(3):175-84. doi: 10.1016/s0268-960x(02)00016-4. PMID: 12163003

Prognosis

Recovery of uninvolved heavy/light chain pair immunoparesis in newly diagnosed transplant-eligible myeloma patients complements the prognostic value of minimal residual disease detection.
Lakhwani S, Rosiñol L, Puig N, Pico-Picos MA, Medina-González L, Martínez-López J, Paiva B, Cedena MT, Oriol A, Ríos-Tamayo R, Blanchard MJ, Jarque I, Bargay J, Moraleda JM, Carrillo-Cruz E, Sureda A, Krsnik I, González E, Casado LF, Martí JM, Encinas C, De Arriba F, Palomera L, Sampol A, González-Montes Y, Motlló C, De La Cruz J, Alonso R, Mateos MV, Bladé J, Lahuerta JJ, San-Miguel J, Hernández MT
Haematologica 2024 Jun 1;109(6):1909-1917. doi: 10.3324/haematol.2023.284154. PMID: 38031761Free PMC Article
Immunoparesis in IgM gammopathies as a useful biomarker to predict disease progression.
Andrade-Campos M, Murillo-Flórez I, García-Sanz R, Giraldo P
Clin Chem Lab Med 2017 Aug 28;55(10):1598-1604. doi: 10.1515/cclm-2016-0748. PMID: 28284031
Benefits of new immunoglobulin-derived biomarkers for the diagnosis and follow-up of patients with dysglobulinemia.
Ermak N, Nguyen-Khoa T, Alyanakian MA
Ann Biol Clin (Paris) 2016 Oct 1;74(5):597-605. doi: 10.1684/abc.2016.1180. PMID: 27707674
T- and B-cell immune reconstitution and clinical outcome in patients with multiple myeloma receiving T-cell-depleted, reduced-intensity allogeneic stem cell transplantation with an alemtuzumab-containing conditioning regimen followed by escalated donor lymphocyte infusions.
D'Sa S, Peggs K, Pizzey A, Verfuerth S, Thuraisundaram D, Watts M, White H, Hale G, Waldmann H, Goldstone A, Mackinnon S, Yong K
Br J Haematol 2003 Oct;123(2):309-22. doi: 10.1046/j.1365-2141.2003.04612.x. PMID: 14531914
Heavy chain disease.
Witzig TE, Wahner-Roedler DL
Curr Treat Options Oncol 2002 Jun;3(3):247-54. doi: 10.1007/s11864-002-0014-3. PMID: 12057070

Clinical prediction guides

Recovery of uninvolved heavy/light chain pair immunoparesis in newly diagnosed transplant-eligible myeloma patients complements the prognostic value of minimal residual disease detection.
Lakhwani S, Rosiñol L, Puig N, Pico-Picos MA, Medina-González L, Martínez-López J, Paiva B, Cedena MT, Oriol A, Ríos-Tamayo R, Blanchard MJ, Jarque I, Bargay J, Moraleda JM, Carrillo-Cruz E, Sureda A, Krsnik I, González E, Casado LF, Martí JM, Encinas C, De Arriba F, Palomera L, Sampol A, González-Montes Y, Motlló C, De La Cruz J, Alonso R, Mateos MV, Bladé J, Lahuerta JJ, San-Miguel J, Hernández MT
Haematologica 2024 Jun 1;109(6):1909-1917. doi: 10.3324/haematol.2023.284154. PMID: 38031761Free PMC Article
The clinical relevance and management of monoclonal gammopathy of undetermined significance and related disorders: recommendations from the European Myeloma Network.
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
Clonal expansion and functional exhaustion of monoclonal marginal zone B cells in mixed cryoglobulinemia: the yin and yang of HCV-driven lymphoproliferation and autoimmunity.
Visentini M, Conti V, Cristofoletti C, Lazzeri C, Marrapodi R, Russo G, Casato M, Fiorilli M
Autoimmun Rev 2013 Jan;12(3):430-5. Epub 2012 Aug 23 doi: 10.1016/j.autrev.2012.08.016. PMID: 22940579
Macular skin lesion and monoclonal lymphoplasmacytoid infiltrates. Occurrence in primary Waldenström's macroglobulinemia.
Mozzanica N, Finzi AF, Facchetti G, Villa ML
Arch Dermatol 1984 Jun;120(6):778-81. PMID: 6426407
Simultaneous occurrence of clinical manifestations of myeloma and Waldenström's macroglobulinemia with monoclonal IgG Lambda and IgM Kappa in a single patient.
Fine JM, Gorin NC, Gendre JP, Petitpierre JC, Labro-Bryskier MT, Lambin P
Acta Med Scand 1981;209(3):229-34. doi: 10.1111/j.0954-6820.1981.tb11582.x. PMID: 6784449

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