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Paraproteinemia

MedGen UID:
10577
•Concept ID:
C0030489
•
Disease or Syndrome
Synonyms: Paraimmunoglobulinemia; Paraimmunoglobulinemias; Paraproteinemias
SNOMED CT: Paraproteinemia (35601003)
 
HPO: HP:0031047

Definition

An abnormal immunoglobulin or part of an Ig (light chain) in the circulation. Paraproteins are typically produced by a clonal population of B-cell derived plasma cells. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVParaproteinemia

Conditions with this feature

Multiple myeloma
MedGen UID:
10122
•Concept ID:
C0026764
•
Neoplastic Process
Multiple myeloma is a neoplastic plasma cell disorder characterized by clonal proliferation of malignant plasma cells in the bone marrow microenvironment, monoclonal protein in the blood or urine, and associated organ dysfunction (Palumbo and Anderson, 2011).
See: Condition Record
Multiple myeloma

Professional guidelines

PubMed

Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review.
Khouri J, Nakashima M, Wong S
JAMA Oncol 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586. PMID: 34081097
What is new in the treatment of Waldenstrom macroglobulinemia?
Castillo JJ, Treon SP
Leukemia 2019 Nov;33(11):2555-2562. Epub 2019 Oct 7 doi: 10.1038/s41375-019-0592-8. PMID: 31591468
Treatment of necrobiotic xanthogranuloma - a systematic review.
Miguel D, Lukacs J, Illing T, Elsner P
J Eur Acad Dermatol Venereol 2017 Feb;31(2):221-235. Epub 2016 Jul 19 doi: 10.1111/jdv.13786. PMID: 27436448

Recent clinical studies

Etiology

Scleromyxedema.
Hoffmann JHO, Enk AH
J Dtsch Dermatol Ges 2020 Dec;18(12):1449-1467. doi: 10.1111/ddg.14319. PMID: 33373143
Paraproteinemic neuropathies.
Raheja D, Specht C, Simmons Z
Muscle Nerve 2015 Jan;51(1):1-13. Epub 2014 Nov 22 doi: 10.1002/mus.24471. PMID: 25288371
Bone and paraproteinemias.
Zangari M, Zhan F, Tricot G
Curr Opin Support Palliat Care 2010 Sep;4(3):195-9. doi: 10.1097/SPC.0b013e32833d3011. PMID: 20657285
Scleromyxedema revisited.
Pomann JJ, Rudner EJ
Int J Dermatol 2003 Jan;42(1):31-5. doi: 10.1046/j.1365-4362.2003.01565.x. PMID: 12581140
Paraproteinemia and neuropathy.
Simmons Z
Curr Opin Neurol 1999 Oct;12(5):589-95. doi: 10.1097/00019052-199910000-00013. PMID: 10590896

Diagnosis

Paraproteinemia and neuropathy.
Koike H, Katsuno M
Neurol Sci 2021 Nov;42(11):4489-4501. Epub 2021 Sep 16 doi: 10.1007/s10072-021-05583-7. PMID: 34529193
Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review.
Khouri J, Nakashima M, Wong S
JAMA Oncol 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586. PMID: 34081097
Scleromyxedema.
Hoffmann JHO, Enk AH
J Dtsch Dermatol Ges 2020 Dec;18(12):1449-1467. doi: 10.1111/ddg.14319. PMID: 33373143
Fibrogenesis Imperfecta Ossium.
Bhadada SK, Dhaliwal R, Dhiman V, Rao SD
Calcif Tissue Int 2019 May;104(5):561-569. Epub 2019 May 8 doi: 10.1007/s00223-019-00547-8. PMID: 31069441
Paraproteinemic neuropathies.
Raheja D, Specht C, Simmons Z
Muscle Nerve 2015 Jan;51(1):1-13. Epub 2014 Nov 22 doi: 10.1002/mus.24471. PMID: 25288371

Therapy

What is new in the treatment of Waldenstrom macroglobulinemia?
Castillo JJ, Treon SP
Leukemia 2019 Nov;33(11):2555-2562. Epub 2019 Oct 7 doi: 10.1038/s41375-019-0592-8. PMID: 31591468
Fibrogenesis Imperfecta Ossium.
Bhadada SK, Dhaliwal R, Dhiman V, Rao SD
Calcif Tissue Int 2019 May;104(5):561-569. Epub 2019 May 8 doi: 10.1007/s00223-019-00547-8. PMID: 31069441
Treatment of necrobiotic xanthogranuloma - a systematic review.
Miguel D, Lukacs J, Illing T, Elsner P
J Eur Acad Dermatol Venereol 2017 Feb;31(2):221-235. Epub 2016 Jul 19 doi: 10.1111/jdv.13786. PMID: 27436448
Bone and paraproteinemias.
Zangari M, Zhan F, Tricot G
Curr Opin Support Palliat Care 2010 Sep;4(3):195-9. doi: 10.1097/SPC.0b013e32833d3011. PMID: 20657285
Scleromyxedema.
Chanda JJ
Cutis 1979 Nov;24(5):549-52. PMID: 520033

Prognosis

Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review.
Khouri J, Nakashima M, Wong S
JAMA Oncol 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586. PMID: 34081097
Scleromyxedema.
Hoffmann JHO, Enk AH
J Dtsch Dermatol Ges 2020 Dec;18(12):1449-1467. doi: 10.1111/ddg.14319. PMID: 33373143
Fibrogenesis Imperfecta Ossium.
Bhadada SK, Dhaliwal R, Dhiman V, Rao SD
Calcif Tissue Int 2019 May;104(5):561-569. Epub 2019 May 8 doi: 10.1007/s00223-019-00547-8. PMID: 31069441
Bone and paraproteinemias.
Zangari M, Zhan F, Tricot G
Curr Opin Support Palliat Care 2010 Sep;4(3):195-9. doi: 10.1097/SPC.0b013e32833d3011. PMID: 20657285
Scleromyxedema.
Chanda JJ
Cutis 1979 Nov;24(5):549-52. PMID: 520033

Clinical prediction guides

Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review.
Khouri J, Nakashima M, Wong S
JAMA Oncol 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586. PMID: 34081097
Fibrogenesis Imperfecta Ossium.
Bhadada SK, Dhaliwal R, Dhiman V, Rao SD
Calcif Tissue Int 2019 May;104(5):561-569. Epub 2019 May 8 doi: 10.1007/s00223-019-00547-8. PMID: 31069441
Paraproteinemic maculopathy.
Mansour AM, Arevalo JF, Badal J, Moorthy RS, Shah GK, Zegarra H, Pulido JS, Charbaji A, Amselem L, Lavaque AJ, Casella A, Ahmad B, Paschall JG, Caimi A, Staurenghi G
Ophthalmology 2014 Oct;121(10):1925-32. Epub 2014 Jun 18 doi: 10.1016/j.ophtha.2014.04.007. PMID: 24950591
Lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia.
Vitolo U, Ferreri AJ, Montoto S
Crit Rev Oncol Hematol 2008 Aug;67(2):172-85. Epub 2008 May 21 doi: 10.1016/j.critrevonc.2008.03.008. PMID: 18499469
Necrobiotic xanthogranuloma.
Fernández-Herrera J, Pedraz J
Semin Cutan Med Surg 2007 Jun;26(2):108-13. doi: 10.1016/j.sder.2007.02.008. PMID: 17544963

Recent systematic reviews

A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria.
Nelson CA, Zhong CS, Hashemi DA, Ashchyan HJ, Brown-Joel Z, Noe MH, Imadojemu S, Micheletti RG, Vleugels RA, Wanat KA, Rosenbach M, Mostaghimi A
JAMA Dermatol 2020 Mar 1;156(3):270-279. doi: 10.1001/jamadermatol.2019.4221. PMID: 31940000Free PMC Article
Treatment of necrobiotic xanthogranuloma - a systematic review.
Miguel D, Lukacs J, Illing T, Elsner P
J Eur Acad Dermatol Venereol 2017 Feb;31(2):221-235. Epub 2016 Jul 19 doi: 10.1111/jdv.13786. PMID: 27436448
Prevalence of monoclonal gammopathy of undetermined significance: a systematic review.
Wadhera RK, Rajkumar SV
Mayo Clin Proc 2010 Oct;85(10):933-42. Epub 2010 Aug 16 doi: 10.4065/mcp.2010.0337. PMID: 20713974Free PMC Article

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