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Polyclonal hypergammaglobulinemia

MedGen UID:
56337
•Concept ID:
C0154254
•
Disease or Syndrome
Synonyms: Polyclonal Hypergammaglobulinemia; polyclonal hypergammaglobulinemia
SNOMED CT: Polyclonal hypergammaglobulinemia (190808009)
 
Monarch Initiative: MONDO:0002272

Definition

A laboratory test result indicating abnormally high proliferation of gamma globulins in the blood originating from multiple cell lines. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPolyclonal hypergammaglobulinemia

Professional guidelines

PubMed

Clinical utility of serum IgG4 measurement.
Varghese JL, Fung AWS, Mattman A, Quach TTT, Gauiran DTV, Carruthers MN, Chen LYC
Clin Chim Acta 2020 Jul;506:228-235. Epub 2020 Apr 6 doi: 10.1016/j.cca.2020.04.001. PMID: 32272158
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.
Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Chadburn A, Colleoni G, Nasta S, Jayanthan R, Nabel CS, Casper C, Dispenzieri A, Fosså A, Kelleher D, Kurzrock R, Voorhees P, Dogan A, Yoshizaki K, van Rhee F, Oksenhendler E, Jaffe ES, Elenitoba-Johnson KS, Lim MS
Blood 2017 Mar 23;129(12):1646-1657. Epub 2017 Jan 13 doi: 10.1182/blood-2016-10-746933. PMID: 28087540Free PMC Article
Evaluation and management of angioimmunoblastic T-cell lymphoma: a review of current approaches and future strategies.
Alizadeh AA, Advani RH
Clin Adv Hematol Oncol 2008 Dec;6(12):899-909. PMID: 19209140

Recent clinical studies

Etiology

Utility of Serum IgG4 Levels in a Multiethnic Population.
Qi R, Chen LYC, Park S, Irvine R, Seidman MA, Kelsall JT, Collins D, Yin V, Slack GW, Mattman A, Lam E, Carruthers MN
Am J Med Sci 2018 Jan;355(1):61-66. Epub 2017 Aug 24 doi: 10.1016/j.amjms.2017.08.014. PMID: 29289265
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.
Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Chadburn A, Colleoni G, Nasta S, Jayanthan R, Nabel CS, Casper C, Dispenzieri A, Fosså A, Kelleher D, Kurzrock R, Voorhees P, Dogan A, Yoshizaki K, van Rhee F, Oksenhendler E, Jaffe ES, Elenitoba-Johnson KS, Lim MS
Blood 2017 Mar 23;129(12):1646-1657. Epub 2017 Jan 13 doi: 10.1182/blood-2016-10-746933. PMID: 28087540Free PMC Article
Multicentric Castleman's disease in HIV infection: a systematic review of the literature.
Mylona EE, Baraboutis IG, Lekakis LJ, Georgiou O, Papastamopoulos V, Skoutelis A
AIDS Rev 2008 Jan-Mar;10(1):25-35. PMID: 18385778
Plasma cell disorders in HIV-infected patients: from benign gammopathy to multiple myeloma.
Dezube BJ, Aboulafia DM, Pantanowitz L
AIDS Read 2004 Jul;14(7):372-4, 377-9. PMID: 15282866
Acquired immune deficiency syndrome in childhood.
Shannon KM, Ammann AJ
J Pediatr 1985 Feb;106(2):332-42. doi: 10.1016/s0022-3476(85)80320-6. PMID: 2982008

Diagnosis

Inflammatory diseases in hematology: a review.
Henrie R, Cherniawsky H, Marcon K, Zhao EJ, Marinkovic A, Pourshahnazari P, Parkin S, Chen LYC
Am J Physiol Cell Physiol 2022 Oct 1;323(4):C1121-C1136. Epub 2022 Aug 8 doi: 10.1152/ajpcell.00356.2021. PMID: 35938681
Clinical utility of serum IgG4 measurement.
Varghese JL, Fung AWS, Mattman A, Quach TTT, Gauiran DTV, Carruthers MN, Chen LYC
Clin Chim Acta 2020 Jul;506:228-235. Epub 2020 Apr 6 doi: 10.1016/j.cca.2020.04.001. PMID: 32272158
IgG4-related disease: what a hematologist needs to know.
Chen LYC, Mattman A, Seidman MA, Carruthers MN
Haematologica 2019 Mar;104(3):444-455. Epub 2019 Jan 31 doi: 10.3324/haematol.2018.205526. PMID: 30705099Free PMC Article
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.
Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Chadburn A, Colleoni G, Nasta S, Jayanthan R, Nabel CS, Casper C, Dispenzieri A, Fosså A, Kelleher D, Kurzrock R, Voorhees P, Dogan A, Yoshizaki K, van Rhee F, Oksenhendler E, Jaffe ES, Elenitoba-Johnson KS, Lim MS
Blood 2017 Mar 23;129(12):1646-1657. Epub 2017 Jan 13 doi: 10.1182/blood-2016-10-746933. PMID: 28087540Free PMC Article
Cutaneous and systemic plasmocytosis.
Wagner G, Rose C, Klapper W, Sachse MM
J Dtsch Dermatol Ges 2013 Dec;11(12):1161-7. Epub 2013 Aug 12 doi: 10.1111/ddg.12190. PMID: 23937389

Therapy

Utility of Serum IgG4 Levels in a Multiethnic Population.
Qi R, Chen LYC, Park S, Irvine R, Seidman MA, Kelsall JT, Collins D, Yin V, Slack GW, Mattman A, Lam E, Carruthers MN
Am J Med Sci 2018 Jan;355(1):61-66. Epub 2017 Aug 24 doi: 10.1016/j.amjms.2017.08.014. PMID: 29289265
Cutaneous and systemic plasmocytosis.
Wagner G, Rose C, Klapper W, Sachse MM
J Dtsch Dermatol Ges 2013 Dec;11(12):1161-7. Epub 2013 Aug 12 doi: 10.1111/ddg.12190. PMID: 23937389
Angioimmunoblastic T-cell lymphoma.
Iannitto E, Ferreri AJ, Minardi V, Tripodo C, Kreipe HH
Crit Rev Oncol Hematol 2008 Dec;68(3):264-71. Epub 2008 Aug 6 doi: 10.1016/j.critrevonc.2008.06.012. PMID: 18684638
Multicentric Castleman's disease in HIV infection: a systematic review of the literature.
Mylona EE, Baraboutis IG, Lekakis LJ, Georgiou O, Papastamopoulos V, Skoutelis A
AIDS Rev 2008 Jan-Mar;10(1):25-35. PMID: 18385778
Plasma cell disorders in HIV-infected patients: from benign gammopathy to multiple myeloma.
Dezube BJ, Aboulafia DM, Pantanowitz L
AIDS Read 2004 Jul;14(7):372-4, 377-9. PMID: 15282866

Prognosis

IgG4-related disease: what a hematologist needs to know.
Chen LYC, Mattman A, Seidman MA, Carruthers MN
Haematologica 2019 Mar;104(3):444-455. Epub 2019 Jan 31 doi: 10.3324/haematol.2018.205526. PMID: 30705099Free PMC Article
Evaluation and management of angioimmunoblastic T-cell lymphoma: a review of current approaches and future strategies.
Alizadeh AA, Advani RH
Clin Adv Hematol Oncol 2008 Dec;6(12):899-909. PMID: 19209140
Angioimmunoblastic T-cell lymphoma.
Iannitto E, Ferreri AJ, Minardi V, Tripodo C, Kreipe HH
Crit Rev Oncol Hematol 2008 Dec;68(3):264-71. Epub 2008 Aug 6 doi: 10.1016/j.critrevonc.2008.06.012. PMID: 18684638
Multicentric Castleman's disease in HIV infection: a systematic review of the literature.
Mylona EE, Baraboutis IG, Lekakis LJ, Georgiou O, Papastamopoulos V, Skoutelis A
AIDS Rev 2008 Jan-Mar;10(1):25-35. PMID: 18385778
Angioimmunoblastic lymphadenopathy with dysproteinemia: emphasis on pathogenesis and treatment.
Sallah S, Gagnon GA
Acta Haematol 1998;99(2):57-64. doi: 10.1159/000040811. PMID: 9554450

Clinical prediction guides

ADA2 Deficiency Mimicking Idiopathic Multicentric Castleman Disease.
Van Nieuwenhove E, Humblet-Baron S, Van Eyck L, De Somer L, Dooley J, Tousseyn T, Hershfield M, Liston A, Wouters C
Pediatrics 2018 Sep;142(3) doi: 10.1542/peds.2017-2266. PMID: 30139808
Cutaneous and systemic plasmocytosis.
Wagner G, Rose C, Klapper W, Sachse MM
J Dtsch Dermatol Ges 2013 Dec;11(12):1161-7. Epub 2013 Aug 12 doi: 10.1111/ddg.12190. PMID: 23937389
B lymphocyte stimulator (BLyS): a therapeutic trichotomy for the treatment of B lymphocyte diseases.
Nardelli B, Moore PA, Li Y, Hilbert DM
Leuk Lymphoma 2002 Jul;43(7):1367-73. doi: 10.1080/10428190290033297. PMID: 12389615
Use of synthetic peptides for the detection and quantification of autoantibodies.
Elkon KB
Mol Biol Rep 1992 Jun;16(3):207-12. doi: 10.1007/BF00464709. PMID: 1508160
Acquired immune deficiency syndrome in childhood.
Shannon KM, Ammann AJ
J Pediatr 1985 Feb;106(2):332-42. doi: 10.1016/s0022-3476(85)80320-6. PMID: 2982008

Recent systematic reviews

Multicentric Castleman's disease in HIV infection: a systematic review of the literature.
Mylona EE, Baraboutis IG, Lekakis LJ, Georgiou O, Papastamopoulos V, Skoutelis A
AIDS Rev 2008 Jan-Mar;10(1):25-35. PMID: 18385778

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