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Lymphadenopathy with Polyclonal Hypergammaglobulinemia

MedGen UID:
233631
•Concept ID:
C1334458
•
Finding

Definition

A finding indicating the presence of enlarged lymph nodes and associated polyclonal hypergammaglobulinemia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLymphadenopathy with Polyclonal Hypergammaglobulinemia

Professional guidelines

PubMed

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
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

Recent clinical studies

Etiology

Idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD-not otherwise specified with different clinical features and better survival.
Gao YH, Liu YT, Zhang MY, Li SY, Fajgenbaum DC, Zhang L, Li J
Br J Haematol 2024 May;204(5):1830-1837. Epub 2024 Feb 15 doi: 10.1111/bjh.19334. PMID: 38356434Free PMC Article
Idiopathic plasmacytic lymphadenopathy: A conceptual history along with a translation of the original Japanese article published in 1980.
Takeuchi K
J Clin Exp Hematop 2022;62(2):79-84. doi: 10.3960/jslrt.22011. PMID: 35768240Free PMC Article
Immunoglobulinopathies in patients with angioimmunoblastic T-cell lymphoma.
Chernova NG, Soboleva NP, Mariina SA, Sidorova YV, Sinitsyna MN, Dvirnyk VN, Badmazhapova DS, Vinogradova YE, Zvonkov EE, Savchenko VG
Ter Arkh 2018 Aug 17;90(7):51-56. doi: 10.26442/terarkh201890751-56. PMID: 30701922
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
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

Diagnosis

Idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD-not otherwise specified with different clinical features and better survival.
Gao YH, Liu YT, Zhang MY, Li SY, Fajgenbaum DC, Zhang L, Li J
Br J Haematol 2024 May;204(5):1830-1837. Epub 2024 Feb 15 doi: 10.1111/bjh.19334. PMID: 38356434Free PMC Article
Morphologic Spectrum of Lymphadenopathy in Drug Reaction With Eosinophilia and Systemic Symptoms Syndrome.
Chen HC, Wang RC, Tsai HP, Medeiros LJ, Chang KC
Arch Pathol Lab Med 2022 Sep 1;146(9):1084-1093. doi: 10.5858/arpa.2021-0087-OA. PMID: 34902854
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
Immunoglobulinopathies in patients with angioimmunoblastic T-cell lymphoma.
Chernova NG, Soboleva NP, Mariina SA, Sidorova YV, Sinitsyna MN, Dvirnyk VN, Badmazhapova DS, Vinogradova YE, Zvonkov EE, Savchenko VG
Ter Arkh 2018 Aug 17;90(7):51-56. doi: 10.26442/terarkh201890751-56. PMID: 30701922
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

Therapy

Utility of renal biopsy in differentiating idiopathic multicentric Castleman disease from IgG4-related disease.
Kawanishi M, Kamei F, Sonoda H, Oba M, Fukunaga S, Egawa M, Koyama T, Sato Y, Tanabe K, Ito T
CEN Case Rep 2023 May;12(2):242-248. Epub 2022 Nov 21 doi: 10.1007/s13730-022-00751-w. PMID: 36414812Free PMC Article
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
Ocular adnexal mucosa-associated lymphoid tissue lymphoma with polyclonal hypergammaglobulinemia.
Kubota T, Moritani S, Yoshino T, Nagai H, Terasaki H
Am J Ophthalmol 2008 Jun;145(6):1002-1006. Epub 2008 Mar 12 doi: 10.1016/j.ajo.2008.01.006. PMID: 18336788
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

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
Immunoglobulinopathies in patients with angioimmunoblastic T-cell lymphoma.
Chernova NG, Soboleva NP, Mariina SA, Sidorova YV, Sinitsyna MN, Dvirnyk VN, Badmazhapova DS, Vinogradova YE, Zvonkov EE, Savchenko VG
Ter Arkh 2018 Aug 17;90(7):51-56. doi: 10.26442/terarkh201890751-56. PMID: 30701922
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

Idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD-not otherwise specified with different clinical features and better survival.
Gao YH, Liu YT, Zhang MY, Li SY, Fajgenbaum DC, Zhang L, Li J
Br J Haematol 2024 May;204(5):1830-1837. Epub 2024 Feb 15 doi: 10.1111/bjh.19334. PMID: 38356434Free PMC Article
Utility of renal biopsy in differentiating idiopathic multicentric Castleman disease from IgG4-related disease.
Kawanishi M, Kamei F, Sonoda H, Oba M, Fukunaga S, Egawa M, Koyama T, Sato Y, Tanabe K, Ito T
CEN Case Rep 2023 May;12(2):242-248. Epub 2022 Nov 21 doi: 10.1007/s13730-022-00751-w. PMID: 36414812Free PMC Article
Active IgG4-related disease with bone marrow involvement: a report of 2 cases and case-based review.
Liu S, Wang H, Su T
Eur J Med Res 2022 Feb 2;27(1):17. doi: 10.1186/s40001-022-00643-w. PMID: 35109941Free PMC Article
Immunoglobulinopathies in patients with angioimmunoblastic T-cell lymphoma.
Chernova NG, Soboleva NP, Mariina SA, Sidorova YV, Sinitsyna MN, Dvirnyk VN, Badmazhapova DS, Vinogradova YE, Zvonkov EE, Savchenko VG
Ter Arkh 2018 Aug 17;90(7):51-56. doi: 10.26442/terarkh201890751-56. PMID: 30701922
Angio-immunoblastic lymphadenopathy. Diagnosis and clinical course.
Frizzera G, Moran EM, Rappaport H
Am J Med 1975 Dec;59(6):803-18. doi: 10.1016/0002-9343(75)90466-0. PMID: 1190254

Recent systematic reviews

Bone marrow findings of idiopathic Multicentric Castleman disease: A histopathologic analysis and systematic literature review.
Belyaeva E, Rubenstein A, Pierson SK, Dalldorf D, Frank D, Lim MS, Fajgenbaum DC
Hematol Oncol 2022 Apr;40(2):191-201. Epub 2022 Feb 18 doi: 10.1002/hon.2969. PMID: 35104370Free 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

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