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Hypogammaglobulinemia

MedGen UID:
88568
•Concept ID:
C0086438
•
Disease or Syndrome
Synonym: Hypogammaglobulinemias
SNOMED CT: Hypogammaglobulinemia (119250001)

Definition

Decreased concentration of the gamma fraction of serum globulin [from SNOMEDCT_US]

Professional guidelines

PubMed

Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: A Work Group Report of the AAAAI Primary Immunodeficiency and Altered Immune Response Committees.
Otani IM, Lehman HK, Jongco AM, Tsao LR, Azar AE, Tarrant TK, Engel E, Walter JE, Truong TQ, Khan DA, Ballow M, Cunningham-Rundles C, Lu H, Kwan M, Barmettler S
J Allergy Clin Immunol 2022 May;149(5):1525-1560. Epub 2022 Feb 14 doi: 10.1016/j.jaci.2022.01.025. PMID: 35176351
Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management.
Yazdani R, Habibi S, Sharifi L, Azizi G, Abolhassani H, Olbrich P, Aghamohammadi A
J Investig Allergol Clin Immunol 2020;30(1):14-34. Epub 2019 Feb 11 doi: 10.18176/jiaci.0388. PMID: 30741636
WHIM syndrome: Immunopathogenesis, treatment and cure strategies.
McDermott DH, Murphy PM
Immunol Rev 2019 Jan;287(1):91-102. doi: 10.1111/imr.12719. PMID: 30565238

Recent clinical studies

Etiology

Common Variable Immunodeficiency.
Remiker A, Bolling K, Verbsky J
Med Clin North Am 2024 Jan;108(1):107-121. Epub 2023 Jul 21 doi: 10.1016/j.mcna.2023.06.012. PMID: 37951645
Nephrotic syndrome: pathophysiology and consequences.
Claudio P, Gabriella M
J Nephrol 2023 Nov;36(8):2179-2190. Epub 2023 Jul 19 doi: 10.1007/s40620-023-01697-7. PMID: 37466816
Hypogammaglobulinemia, late-onset neutropenia, and infections following rituximab.
Athni TS, Barmettler S
Ann Allergy Asthma Immunol 2023 Jun;130(6):699-712. Epub 2023 Jan 24 doi: 10.1016/j.anai.2023.01.018. PMID: 36706910Free PMC Article
Toxicities following CAR-T therapy for hematological malignancies.
Hernani R, Benzaquén A, Solano C
Cancer Treat Rev 2022 Dec;111:102479. Epub 2022 Oct 22 doi: 10.1016/j.ctrv.2022.102479. PMID: 36308910
Late Events after Treatment with CD19-Targeted Chimeric Antigen Receptor Modified T Cells.
Cordeiro A, Bezerra ED, Hirayama AV, Hill JA, Wu QV, Voutsinas J, Sorror ML, Turtle CJ, Maloney DG, Bar M
Biol Blood Marrow Transplant 2020 Jan;26(1):26-33. Epub 2019 Aug 13 doi: 10.1016/j.bbmt.2019.08.003. PMID: 31419568Free PMC Article

Diagnosis

Common Variable Immunodeficiency.
Remiker A, Bolling K, Verbsky J
Med Clin North Am 2024 Jan;108(1):107-121. Epub 2023 Jul 21 doi: 10.1016/j.mcna.2023.06.012. PMID: 37951645
"Common variable immunodeficiency: Challenges for diagnosis".
Wang LA, Abbott JK
J Immunol Methods 2022 Oct;509:113342. Epub 2022 Aug 24 doi: 10.1016/j.jim.2022.113342. PMID: 36027932
Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management.
Yazdani R, Habibi S, Sharifi L, Azizi G, Abolhassani H, Olbrich P, Aghamohammadi A
J Investig Allergol Clin Immunol 2020;30(1):14-34. Epub 2019 Feb 11 doi: 10.18176/jiaci.0388. PMID: 30741636
X-linked Agammaglobulinemia.
Suri D, Rawat A, Singh S
Indian J Pediatr 2016 Apr;83(4):331-7. Epub 2016 Feb 24 doi: 10.1007/s12098-015-2024-8. PMID: 26909497
Common variable immunodeficiency.
Bonilla FA, Geha RS
Pediatr Res 2009 May;65(5 Pt 2):13R-19R. doi: 10.1203/PDR.0b013e31819dbf88. PMID: 19190529

Therapy

Bispecific antibody therapies.
de Assis LH, Fassi DE, Hutchings M
Hematology Am Soc Hematol Educ Program 2023 Dec 8;2023(1):216-222. doi: 10.1182/hematology.2023000508. PMID: 38066890Free PMC Article
Hypogammaglobulinemia, late-onset neutropenia, and infections following rituximab.
Athni TS, Barmettler S
Ann Allergy Asthma Immunol 2023 Jun;130(6):699-712. Epub 2023 Jan 24 doi: 10.1016/j.anai.2023.01.018. PMID: 36706910Free PMC Article
Hypogammaglobulinemia in pediatric kidney transplant recipients.
Dimitriades V, Butani L
Pediatr Nephrol 2023 Jun;38(6):1753-1762. Epub 2022 Sep 30 doi: 10.1007/s00467-022-05757-1. PMID: 36178549Free PMC Article
Toxicities following CAR-T therapy for hematological malignancies.
Hernani R, Benzaquén A, Solano C
Cancer Treat Rev 2022 Dec;111:102479. Epub 2022 Oct 22 doi: 10.1016/j.ctrv.2022.102479. PMID: 36308910
Hypogammaglobulinemia after rituximab therapy in children.
Newman EN, Israelsen RB, Williamson K, Hsieh EWY
Ann Allergy Asthma Immunol 2022 Feb;128(2):225-226. Epub 2021 Oct 30 doi: 10.1016/j.anai.2021.10.028. PMID: 34728346

Prognosis

Management of chimeric antigen receptor T (CAR-T) cell-associated toxicities.
Schroeder T, Martens T, Fransecky L, Valerius T, Schub N, Pott C, Baldus C, Stölzel F
Intensive Care Med 2024 Sep;50(9):1459-1469. Epub 2024 Aug 22 doi: 10.1007/s00134-024-07576-4. PMID: 39172238Free PMC Article
Long-Term Follow-Up of Anti-CD19 Chimeric Antigen Receptor T-Cell Therapy.
Cappell KM, Sherry RM, Yang JC, Goff SL, Vanasse DA, McIntyre L, Rosenberg SA, Kochenderfer JN
J Clin Oncol 2020 Nov 10;38(32):3805-3815. Epub 2020 Oct 6 doi: 10.1200/JCO.20.01467. PMID: 33021872Free PMC Article
Late Events after Treatment with CD19-Targeted Chimeric Antigen Receptor Modified T Cells.
Cordeiro A, Bezerra ED, Hirayama AV, Hill JA, Wu QV, Voutsinas J, Sorror ML, Turtle CJ, Maloney DG, Bar M
Biol Blood Marrow Transplant 2020 Jan;26(1):26-33. Epub 2019 Aug 13 doi: 10.1016/j.bbmt.2019.08.003. PMID: 31419568Free PMC Article
Common variable immunodeficiency.
Sneller MC
Am J Med Sci 2001 Jan;321(1):42-8. doi: 10.1097/00000441-200101000-00007. PMID: 11202479
Bacterial arthritis.
Ike RW
Curr Opin Rheumatol 1998 Jul;10(4):330-4. doi: 10.1097/00002281-199807000-00009. PMID: 9725094

Clinical prediction guides

Patterns of CMV infection after letermovir withdrawal in recipients of posttransplant cyclophosphamide-based transplant.
Lin A, Brown S, Chinapen S, Lee YJ, Seo SK, Ponce DM, Shahid Z, Giralt S, Papanicolaou GA, Perales MA, Shaffer BC
Blood Adv 2023 Dec 12;7(23):7153-7160. doi: 10.1182/bloodadvances.2023010966. PMID: 37906513Free PMC Article
Advanced computational analysis of CD40LG variants in atypical X-linked hyper-IgM syndrome.
Pazhanisamy A, Jorge SD, Zimmermann MT, Kitcharoensakkul M, Abdalgani M, Khojah A, Victor C, Rueda C, Urrutia R, Abraham RS
Clin Immunol 2023 Aug;253:109692. Epub 2023 Jul 9 doi: 10.1016/j.clim.2023.109692. PMID: 37433422
Use of Rituximab in Childhood Idiopathic Nephrotic Syndrome.
Chan EY, Yap DY, Colucci M, Ma AL, Parekh RS, Tullus K
Clin J Am Soc Nephrol 2023 Apr 1;18(4):533-548. Epub 2023 Feb 22 doi: 10.2215/CJN.08570722. PMID: 36456193Free PMC Article
Late Events after Treatment with CD19-Targeted Chimeric Antigen Receptor Modified T Cells.
Cordeiro A, Bezerra ED, Hirayama AV, Hill JA, Wu QV, Voutsinas J, Sorror ML, Turtle CJ, Maloney DG, Bar M
Biol Blood Marrow Transplant 2020 Jan;26(1):26-33. Epub 2019 Aug 13 doi: 10.1016/j.bbmt.2019.08.003. PMID: 31419568Free PMC Article
Diagnosis and management of ADA2 deficient polyarteritis nodosa.
Human A, Pagnoux C
Int J Rheum Dis 2019 Jan;22 Suppl 1:69-77. Epub 2018 Apr 6 doi: 10.1111/1756-185X.13283. PMID: 29624883

Recent systematic reviews

Progressive multifocal leukoencephalopathy in multiple myeloma: a case report of a patient with SARS-CoV-2 infection and an updated systematic literature review.
Paolucci M, Gentile L, Gentile M, Borghi A, Merli E, Marchionni E, Guerra L, Galluzzo S, Cilloni N, Simonetti L, Zini A
Neurol Sci 2023 Sep;44(9):2995-2998. Epub 2023 Jul 8 doi: 10.1007/s10072-023-06944-0. PMID: 37421487
Clinical Courses of IKAROS and CTLA4 Deficiencies: A Systematic Literature Review and Retrospective Longitudinal Study.
Hoshino A, Toyofuku E, Mitsuiki N, Yamashita M, Okamoto K, Yamamoto M, Kanda K, Yamato G, Keino D, Yoshimoto-Suzuki Y, Kamizono J, Onoe Y, Ichimura T, Nagao M, Yoshimura M, Tsugawa K, Igarashi T, Mitsui-Sekinaka K, Sekinaka Y, Doi T, Yasumi T, Nakazawa Y, Takagi M, Imai K, Nonoyama S, Morio T, Latour S, Kanegane H
Front Immunol 2021;12:784901. Epub 2022 Jan 11 doi: 10.3389/fimmu.2021.784901. PMID: 35087518Free PMC Article
Autoimmunity in common variable immunodeficiency: a systematic review and meta-analysis.
Rizvi FS, Zainaldain H, Rafiemanesh H, Jamee M, Hossein-Khannazer N, Hamedifar H, Sabzevari A, Yazdani R, Abolhassani H, Aghamohammadi A, Azizi G
Expert Rev Clin Immunol 2020 Dec;16(12):1227-1235. Epub 2020 Dec 7 doi: 10.1080/1744666X.2021.1850272. PMID: 33203275
Impact of rare diseases in oral health.
Molina-García A, Castellanos-Cosano L, Machuca-Portillo G, Posada-de la Paz M
Med Oral Patol Oral Cir Bucal 2016 Sep 1;21(5):e587-94. doi: 10.4317/medoral.20972. PMID: 27475682Free PMC Article
Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence.
Kelesidis T, Yang O
Clin Immunol 2010 Jun;135(3):347-63. Epub 2010 Feb 10 doi: 10.1016/j.clim.2010.01.006. PMID: 20149753Free PMC Article

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