paraproteinemia (monoclonal gammopathy)
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Etiology
- plasma cell disorder
- benign monoclonal gammopathy (MGUS)
- biclonal gammopathy of undetermined significance
- idiopathic Bence Jones proteinuria
- POEMS syndrome
- Castleman's disease
- AL amyloidosis, light chain amyloidosis
- solitary plasmacytoma
- multiple myeloma
- monoclonal immunoglobulin deposition disease
- proximal tubulopathy (MGUS, multiple myeloma)
- B-cell proliferative disorder
- connective tissue disease
- infection
- skin disorders
- scleredema, lichen myxoedematosus
- diffuse plane xanthomatosis
- Schnitzler's syndrome
- subcorneal pustular dermatosis
- necrobiotic xanthogranuloma
- pyoderma gangrenosum
- other
- acquired von Willebrand disease
- acquired C1 esterase deficiency
- eosinophilic fasciitis
- cryoglobulinemia
- myelodysplastic syndrome
- chronic myelocytic leukemia
- capillary leak syndrome
- T-cell leukemia, large granular lymphocytic
Pathology
- organized glomerular immune deposition
- AL amyloid
- immunotactoid glomerulonephritis
- fibrillary glomerulonephritis
- cyoglobulinemia
- disorganized glomerular immune deposition (no beta-pleated sheets)
Laboratory
- complete blood count (CBC)
- urinalysis with 24 hour urine protein
- complete metabolic panel for Ca+2, phosphorous
- immunoglobulin light chains.free in serum*
- most sensitive test for free light chain paraproteinemia[4]
- serum protein electrophoresis*
- urine protein electrophoresis
- beta-2 microglobulin in serum
* combination has sensitivity of ~100% diagnosing multiple myeloma requiring therapy
Diagnostic procedures
- bone marrow biopsy not necessary if monoclonal IgG < 1.5 g/dL & no end organ damage (renal function normal), i.e. MGUS
Radiology
- whole body MRI for evaluation of smoldering multiple myeloma
- bone scan not recommended[4]
- multiple myeloma lesions are often purely lytic
- a bone scan detects osteoblastic lesions
More general terms
More specific terms
- benign monoclonal gammopathy; monoclonal gammopathy of undetermined significance (MGUS)
- cryoglobulinemia (cryoglobulinemic vasculitis)
- heavy chain disease; Franklin disease
- monoclonal gammopathy of renal significance
- monoclonal gammopathy/neuropathy syndrome
- monoclonal immunoglobulin deposition disease (MIDD)
- multiple myeloma; plasmacytoma/plasma cell myeloma
- POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, & Skin changes); Crow-Fukase syndrome
- Waldenstrom's macroglobulinemia
References
- ↑ Bataille R, Harousseau JL. Multiple myeloma. N Engl J Med. 1997 Jun 5;336(23):1657-64. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/9171069
- ↑ Veterans Administration, Mather CA
- ↑ UpToDate 14.1 (2007) http://www.utdol.com
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 Medical Knowledge Self Assessment Program (MKSAP) 17, 18, 19. American College of Physicians, Philadelphia 2015, 2018, 2022
- ↑ Katzmann JA, Kyle RA, Benson J et al Screening panels for detection of monoclonal gammopathies. Clin Chem. 2009 Aug;55(8):1517-22 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19520758
- ↑ Zivkovic SA, Lacomis D, Lentzsch S. Paraproteinemic neuropathy. Leuk Lymphoma. 2009 Sep;50(9):1422-33 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19637090
- ↑ Rison RA, Beydoun SR. Paraproteinemic neuropathy: a practical review. BMC Neurol. 2016 Jan 28;16:13. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26821540 Free PMC Article
- ↑ Rosner MH, Edeani A, Yanagita M et al Paraprotein-Related Kidney Disease: Diagnosing and Treating Monoclonal Gammopathy of Renal Significance. Clin J Am Soc Nephrol. 2016 Dec 7;11(12):2280-2287. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27526705 Free PMC Article