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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

Paraprotein-Mediated Glomerular Diseases.
Miao J, Herrmann SM, Obaidi Z, Caza T, Bonilla M
Adv Kidney Dis Health 2024 Jul;31(4):358-373. doi: 10.1053/j.akdh.2024.02.005. PMID: 39084761
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

Paraprotein-Mediated Glomerular Diseases.
Miao J, Herrmann SM, Obaidi Z, Caza T, Bonilla M
Adv Kidney Dis Health 2024 Jul;31(4):358-373. doi: 10.1053/j.akdh.2024.02.005. PMID: 39084761
Paraproteinemias and Peripheral Nerve Disease.
Leavell Y, Shin SC
Clin Geriatr Med 2021 May;37(2):301-312. doi: 10.1016/j.cger.2021.01.004. PMID: 33858612
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
Paraproteinemia and serum protein electrophoresis interpretation.
Raj S, Guha B, Rodriguez C, Krishnaswamy G
Ann Allergy Asthma Immunol 2019 Jan;122(1):11-16. doi: 10.1016/j.anai.2018.08.004. PMID: 30579431
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

Bing-Neel syndrome with a paravertebral mass.
Suzuki D, Sato D, Kikuchi K, Suzuki T, Sugasawa K, Saito S, Uchimura F, Suzuki Y
Am J Med Sci 2023 Oct;366(4):316-320. Epub 2023 Jul 18 doi: 10.1016/j.amjms.2023.07.004. PMID: 37473959
Case report: Scleromyxedema associated with a monoclonal gammapathy: Successful treatment with intravenous immunoglobulins.
Wang SS, Chen QY, Xiang LH
Front Immunol 2022;13:1099918. Epub 2023 Jan 13 doi: 10.3389/fimmu.2022.1099918. PMID: 36713453Free PMC Article
Scleromyxedema without paraproteinemia.
Abarzúa AA, Giesen LF, Sandoval MO, González SB
Int J Dermatol 2014 Aug;53(8):971-4. Epub 2014 Feb 14 doi: 10.1111/ijd.12124. PMID: 24527753
Hematopoietic cell transplantation for Waldenström macroglobulinemia.
Bachanova V, Burns LJ
Bone Marrow Transplant 2012 Mar;47(3):330-6. Epub 2011 May 16 doi: 10.1038/bmt.2011.105. PMID: 21572463
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

Prognosis

POEMS syndrome: A multisystem clonal disorder.
Ali T, Qazilbash MH
Eur J Haematol 2021 Jan;106(1):14-18. Epub 2020 Oct 8 doi: 10.1111/ejh.13514. PMID: 32889731
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
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

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
Scleromyxedema without paraproteinemia.
Abarzúa AA, Giesen LF, Sandoval MO, González SB
Int J Dermatol 2014 Aug;53(8):971-4. Epub 2014 Feb 14 doi: 10.1111/ijd.12124. PMID: 24527753
Hematopoietic cell transplantation for Waldenström macroglobulinemia.
Bachanova V, Burns LJ
Bone Marrow Transplant 2012 Mar;47(3):330-6. Epub 2011 May 16 doi: 10.1038/bmt.2011.105. PMID: 21572463
Rheumatic manifestations of neoplasia.
Brooks PM
Curr Opin Rheumatol 1992 Feb;4(1):90-3. PMID: 1543670

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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