Jump to content

Monoclonal gammopathy of undetermined significance: Difference between revisions

From Wikipedia, the free encyclopedia
Browse history interactively
← Previous editNext edit →
Content deleted Content added
VisualWikitext
→‎Diagnosis: ststed 'following three criteria', yet 4 criteria were listed
Line 21: Line 21:
MGUS is a common, age-related medical condition characterized by an accumulation of bone marrow plasma cells derived from a single abnormal clone. Patients may be diagnosed with MGUS if they fulfill the following four criteria:<ref>{{cite journal | author =International Myeloma Working Group | title = Criteria for the classification of monoclonal gammopathies, [[multiple myeloma]] and related disorders: a report of the International Myeloma Working Group | journal = Br J Haematol | volume = 121 | issue = 5 | pages = 749–757 | year = 2003 | pmid = 12780789 | doi = 10.1046/j.1365-2141.2003.04355.x}}</ref>
MGUS is a common, age-related medical condition characterized by an accumulation of bone marrow plasma cells derived from a single abnormal clone. Patients may be diagnosed with MGUS if they fulfill the following four criteria:<ref>{{cite journal | author =International Myeloma Working Group | title = Criteria for the classification of monoclonal gammopathies, [[multiple myeloma]] and related disorders: a report of the International Myeloma Working Group | journal = Br J Haematol | volume = 121 | issue = 5 | pages = 749–757 | year = 2003 | pmid = 12780789 | doi = 10.1046/j.1365-2141.2003.04355.x}}</ref>


#A monoclonal paraprotein band greater than 30 g/L (> 3g/dL);
#A monoclonal paraprotein band less than 30 g/L (< 3g/dL);
#[[Plasma cell]]s less than 10% on [[bone marrow examination]];
#[[Plasma cell]]s less than 10% on [[bone marrow examination]];
#No evidence of bone lesions, [[anemia]], [[hypercalcemia]], or [[renal insufficiency]] related to the paraprotein, and
#No evidence of bone lesions, [[anemia]], [[hypercalcemia]], or [[renal insufficiency]] related to the paraprotein, and

Revision as of 22:21, 10 August 2013

Monoclonal gammopathy of undetermined significance

Monoclonal gammopathy of undetermined significance (MGUS , unknown or uncertain may be substituted for undetermined), formerly benign monoclonal gammopathy, is a condition in which a paraprotein is found in the blood during standard laboratory tests. It resembles multiple myeloma and similar diseases, but the levels of antibody are lower, the number of plasma cells (white blood cells that secrete antibodies) in the bone marrow is lower, it has no symptoms or problems, and no treatment is indicated. However, multiple myeloma develops at the rate of about 1-2% a year, so doctors recommend monitoring it yearly. In rare cases, it may also be related with a slowly progressive symmetric distal sensorimotor neuropathy.

Diagnosis

MGUS is a common, age-related medical condition characterized by an accumulation of bone marrow plasma cells derived from a single abnormal clone. Patients may be diagnosed with MGUS if they fulfill the following four criteria:[1]

  1. A monoclonal paraprotein band less than 30 g/L (< 3g/dL);
  2. Plasma cells less than 10% on bone marrow examination;
  3. No evidence of bone lesions, anemia, hypercalcemia, or renal insufficiency related to the paraprotein, and
  4. No evidence of another B-cell proliferative disorder.

Differential diagnosis

Several other illnesses can present with a monoclonal gammopathy, and the monoclonal protein may be the first discovery before a formal diagnosis is made:

Pathology

Pathologically, the lesion in MGUS is in fact very similar to that in multiple myeloma. There is a predominance of clonal plasma cells in the bone marrow with an abnormal immunophenotype (CD38+ CD56+ CD19−) mixed in with cells of a normal phenotype (CD38+ CD56− CD19+);[5][6] in MGUS, on average more than 3% of the clonal plasma cells have the normal phenotype, whereas in multiple myeloma, less than 3% of the cells have the normal phenotype.[7] What causes MGUS to transform into multiple myeloma is as yet unknown.

Prognosis

At the Mayo Clinic, MGUS transformed into multiple myeloma or similar myeloproliferative disorder at the rate of about 1-2% a year, or 17%, 34%, and 39% at 10, 20, and 25 years, respectively, of follow-up—among surviving patients. However, because they were elderly, most patients with MGUS died of something else and did not go on to develop multiple myeloma. When this was taken into account, only 11.2% developed myeloproliferative disorders.[8]

Kyle studied the prevalence of myeloma in the population as a whole (not clinic patients) in Olmsted County, Minnesota. They found that the prevalence of MGUS was 3.2% in people above 50, with a slight male predominance (4.0% vs. 2.7%). Prevalence increased with age: of people over 70 up to 5.3% had MGUS, while in the over-85 age group the prevalence was 7.5%. In the majority of cases (63.5%), the paraprotein level was <1 g/dl, while only a very small group had levels over 2 g/dl.[9] A study of monoclonal protein levels conducted in Ghana showed a prevalence of MGUS of approximately 5.9% in African men over the age of 50.[10]

In 2009, prospective data demonstrated that all or almost all cases of Multiple Myeloma are preceded by MGUS.[11] In addition to multiple myeloma, MGUS may also progress to Waldenström's macroglobulinemia, primary amyloidosis, B-cell lymphoma, or chronic lymphocytic leukemia.

Management

The protein electrophoresis test should be repeated annually, and if there is any concern for a rise in the level of monoclonal protein, then prompt referral to a hematologist is required. The hematologist, when first evaluating a case of MGUS, will usually perform a skeletal survey (X-rays of the proximal skeleton), check the blood for hypercalcemia and deterioration in renal function, check the urine for Bence Jones protein and perform a bone marrow biopsy. If none of these tests are abnormal, a patient with MGUS is followed up once every 6 months to a year with a blood test (serum protein electrophoresis).

See also

References

  1. ^ International Myeloma Working Group (2003). "Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group". Br J Haematol. 121 (5): 749–757. doi:10.1046/j.1365-2141.2003.04355.x. PMID 12780789.
  2. ^ Murakami H, Irisawa H, Saitoh T, Matsushima T, Tamura J, Sawamura M, Karasawa M, Hosomura Y, Kojima M (1997). "Immunological abnormalities in splenic marginal zone cell lymphoma". Am. J. Hematol. 56 (3): 173–178. doi:10.1002/(SICI)1096-8652(199711)56:3<173::AID-AJH7>3.0.CO;2-V. PMID 9371530.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  3. ^ Larking-Pettigrew M, Ranich T, Kelly R (1999). "Rapid onset monoclonal gammopathy in cutaneous lupus erythematosus: interference with complement C3 and C4 measurement". Immunol. Invest. 28 (4): 269–276. doi:10.3109/08820139909060861. PMID 10454004.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  4. ^ Czaplinski A, Steck A (2004). "Immune mediated neuropathies--an update on therapeutic strategies". J. Neurol. 251 (2): 127–137. doi:10.1007/s00415-004-0323-5. PMID 14991345.
  5. ^ Zhan F, Hardin J, Kordsmeier B, Bumm K, Zheng M, Tian E, Sanderson R, Yang Y, Wilson C, Zangari M, Anaissie E, Morris C, Muwalla F, van Rhee F, Fassas A, Crowley J, Tricot G, Barlogie B, Shaughnessy J (2002). "Global gene expression profiling of multiple myeloma, monoclonal gammopathy of undetermined significance, and normal bone marrow plasma cells". Blood. 99 (5): 1745–1757. doi:10.1182/blood.V99.5.1745. PMID 11861292.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  6. ^ Magrangeas F, Nasser V, Avet-Loiseau H, Loriod B, Decaux O, Granjeaud S, Bertucci F, Birnbaum D, Nguyen C, Harousseau J, Bataille R, Houlgatte R, Minvielle S (2003). "Gene expression profiling of multiple myeloma reveals molecular portraits in relation to the pathogenesis of the disease". Blood. 101 (12): 4998–5006. doi:10.1182/blood-2002-11-3385. PMID 12623842.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  7. ^ Ocqueteau M, Orfao A, Almeida J, Bladé J, González M, García-Sanz R, López-Berges C, Moro M, Hernández J, Escribano L, Caballero D, Rozman M, San Miguel J (1998). "Immunophenotypic characterization of plasma cells from monoclonal gammopathy of undetermined significance patients. Implications for the differential diagnosis between MGUS and multiple myeloma". Am J Pathol. 152 (6): 1655–65. PMC 1858455. PMID 9626070.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  8. ^ Bladé J (2006). "Clinical practice. Monoclonal gammopathy of undetermined significance". N Engl J Med. 355 (26): 2765–2770. doi:10.1056/NEJMcp052790. PMID 17192542.
  9. ^ Kyle RA, Therneau TM, Rajkumar SV, Larson DR, Plevak MF, Offord JR, Dispenzieri A, Katzmann JA, Melton LJ 3rd. (2006). "Prevalence of monoclonal gammopathy of undetermined significance". N Engl J Med. 354 (13): 1362–1369. doi:10.1056/NEJMoa054494. PMID 16571879. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link) CS1 maint: numeric names: authors list (link)
  10. ^ Landgren O, Katzmann JA, Hsing AW, Pfeiffer RM, Kyle RA, Yeboah ED, Biritwum RB, Tettey Y, Adjei AA, Larson DR, Dispenzieri A, Melton LJ 3rd, Goldin LR, McMaster ML, Caporaso NE, Rajkumar SV. (2007). "Prevalence of monoclonal gammopathy of undetermined significance among men in Ghana". Mayo Clin Proc. 82 (12): 1468–1473. doi:10.4065/82.12.1468. PMID 18053453. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link) CS1 maint: numeric names: authors list (link)
  11. ^ Landgren O, Kyle RA, Pfeiffer RM, Katzmann JA, Caporaso NE, Hayes RB, Dispenzieri A, Kumar S, Clark RJ, Baris D, Hoover R, Rajkumar SV. (2009). "Monoclonal gammopathy of undetermined significance (MGUS) consistently precedes multiple myeloma: a prospective study". Blood. 113 (22): 5412–7. doi:10.1182/blood-2008-12-194241. PMC 2689042. PMID 19179464. {{cite journal}}: Unknown parameter |month= ignored (help); Unknown parameter |unused_data= ignored (help)CS1 maint: multiple names: authors list (link)

Further reading

  • Advances in understanding monoclonal gammopathy of undetermined significance as a precursor of multiple myeloma.

Brendan W. Weiss & Michael Kuehl. Expert Rev Hematol. 3(2), 165-174 (2010)

  • New criteria to identify risk of progression in monoclonal gammopathy of uncertain significance and smoldering multiple

myeloma based on multiparameter flow cytometry analisis of bone marrow plasma cells. Ernesto Perez-Persona et al. Blood, 1 October 2007. Vol 110. N 7, 2586–2592

  • Seven-year median time to progression with thalidomide for smoldering myeloma: partial response identifies subset requiring

earlier salvage therapy for symptomatic disease. Bart Barlogie et al. Blood 15 October 2008. Vol 112. N 8, 3122-3125

  • Proposal and Validation of Prognostic Scoring Systems for IgG and IgA Monoclonal Gammopathies of Undetermined Significance.

Francesca Rossi et al. Clin Cancer Res 2009; 15 (13), July 1, 2009, 4439-4445

  • The potential Role of Curcumin in Patients with Monoclonal Gammopathy of Undetermined Significance-Its effect on

Paraproteinemia and the Urinary N-Telopeptide of Type I Collagen Bone Turnover Marker. Terry Golombick et al. Clin Cancer Res 2009; 15(18), September 15, 2009, 5917-5922

  • Prevention of Progression in Monoclonal Gammopathy of Undetermined Significance. S. Vincent Rajkumar. Clin Cancer

Res 2009; 15(18), September 15, 2009, 5606-5608

  • Monoclonal Gammopathy of Undetermined Significance: a consensus statement. James R. Berenson et al. Brit J Hematol, 2010, 150, 28-38
  • Monoclonal Gammopathy of Undetermined Significance (MGUS) and Smoldering (asymptomatic) Multile Myeloma: IMWG consensus

perspectives risk factors for progression and Guidelines for monitoring and management. R A Kyle et al. Leukemia (2010, 1-7

Retrieved from "https://en.wikipedia.org/w/index.php?title=Monoclonal_gammopathy_of_undetermined_significance&oldid=568001762"