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Impaired memory B cell generation

MedGen UID:
871195
Concept ID:
C4025672
Cell or Molecular Dysfunction
HPO: HP:0002847

Definition

Impaired production of memory cells, the B cells that persist for years or an entire lifetime and which confer rapid and enhanced response to secondary challenge. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVImpaired memory B cell generation

Conditions with this feature

Hyper-IgM syndrome type 1
MedGen UID:
96019
Concept ID:
C0398689
Disease or Syndrome
X-linked hyper IgM syndrome (HIGM1), a disorder of abnormal T- and B-cell function, is characterized by low serum concentrations of IgG, IgA, and IgE with normal or elevated serum concentrations of IgM. Mitogen proliferation may be normal, but NK- and T-cell cytotoxicity can be impaired. Antigen-specific responses are usually decreased or absent. Total numbers of B cells are normal but there is a marked reduction of class-switched memory B cells. Defective oxidative burst of both neutrophils and macrophages has been reported. The range of clinical findings varies, even within the same family. More than 50% of males with HIGM1 develop symptoms by age one year, and more than 90% are symptomatic by age four years. HIGM1 usually presents in infancy with recurrent upper- and lower-respiratory tract bacterial infections, opportunistic infections including Pneumocystis jirovecii pneumonia, and recurrent or protracted diarrhea that can be infectious or noninfectious and is associated with failure to thrive. Neutropenia is common; thrombocytopenia and anemia are less commonly seen. Autoimmune and/or inflammatory disorders (such as sclerosing cholangitis) as well as increased risk for neoplasms have been reported as medical complications of this disorder. Significant neurologic complications, often the result of a CNS infection, are seen in 5%-15% of affected males. Liver disease, a serious complication of HIGM1 once observed in more than 80% of affected males by age 20 years, may be decreasing with adequate screening and treatment of Cryptosporidium infection.
Hyper-IgM syndrome type 3
MedGen UID:
328419
Concept ID:
C1720957
Disease or Syndrome
Type 3 immunodeficiency with hyper-IgM (HIGM3), first described in humans by Ferrari et al. (2001), is characterized by hypogammaglobulinemia with normal or elevated levels of IgM. For a general phenotypic description and a discussion of genetic heterogeneity of immunodeficiency with hyper-IgM, see HIGM1 (308230).

Recent clinical studies

Etiology

Monaco MCG, Soldan SS, Su C, Clauze A, Cooper JF, Patel RJ, Lu F, Hughes RJ, Messick TE, Andrada FC, Ohayon J, Lieberman PM, Jacobson S
Neurol Neuroimmunol Neuroinflamm 2023 Sep;10(5) Epub 2023 Aug 10 doi: 10.1212/NXI.0000000000200149. PMID: 37562974Free PMC Article
Abd Alhadi M, Friedman LM, Karlsson EA, Cohen-Lavi L, Burkovitz A, Schultz-Cherry S, Noah TL, Weir SS, Shulman LM, Beck MA, Hertz T
Microbiol Spectr 2023 Jun 15;11(3):e0001023. Epub 2023 Apr 26 doi: 10.1128/spectrum.00010-23. PMID: 37098954Free PMC Article
Werner L, Dor C, Salamon N, Nagar M, Shouval DS
J Vis Exp 2021 Jan 12;(167) doi: 10.3791/61792. PMID: 33522509
Roos-Weil D, Giacopelli B, Armand M, Della-Valle V, Ghamlouch H, Decaudin C, Metzner M, Lu J, Le Garff-Tavernier M, Leblond V, Vyas P, Zenz T, Nguyen-Khac F, Bernard OA, Oakes CC
Blood 2020 Jul 30;136(5):585-595. doi: 10.1182/blood.2020005081. PMID: 32457988
Perez-Andres M, Paiva B, Nieto WG, Caraux A, Schmitz A, Almeida J, Vogt RF Jr, Marti GE, Rawstron AC, Van Zelm MC, Van Dongen JJ, Johnsen HE, Klein B, Orfao A; Primary Health Care Group of Salamanca for the Study of MBL
Cytometry B Clin Cytom 2010;78 Suppl 1:S47-60. doi: 10.1002/cyto.b.20547. PMID: 20839338

Diagnosis

van Schouwenburg P, Unger S, Payne KJ, Kaiser FMP, Pico-Knijnenburg I, Pfeiffer J, Hausmann O, Friedmann D, Erbel M, Seidl M, van Zessen D, Stubbs AP, van der Burg M, Warnatz K
Front Immunol 2022;13:959002. Epub 2022 Oct 6 doi: 10.3389/fimmu.2022.959002. PMID: 36275744Free PMC Article
Rodríguez-Ubreva J, Arutyunyan A, Bonder MJ, Del Pino-Molina L, Clark SJ, de la Calle-Fabregat C, Garcia-Alonso L, Handfield LF, Ciudad L, Andrés-León E, Krueger F, Català-Moll F, Rodríguez-Cortez VC, Polanski K, Mamanova L, van Dongen S, Kiselev VY, Martínez-Saavedra MT, Heyn H, Martín J, Warnatz K, López-Granados E, Rodríguez-Gallego C, Stegle O, Kelsey G, Vento-Tormo R, Ballestar E
Nat Commun 2022 Apr 1;13(1):1779. doi: 10.1038/s41467-022-29450-x. PMID: 35365635Free PMC Article
AbdelRazek MA, Casasola M, Mollashahi R, Brodski A, Morin S, Augustynowicz A, Jassim S, Matiello M, Sloane J
Mult Scler Relat Disord 2022 Mar;59:103505. Epub 2022 Jan 21 doi: 10.1016/j.msard.2022.103505. PMID: 35121247
Fernando SL, Jang HS, Li J
Immunol Lett 2021 Feb;230:21-26. Epub 2020 Dec 14 doi: 10.1016/j.imlet.2020.12.002. PMID: 33333111
Farmer JR, DeLelys M
Clin Lab Med 2019 Dec;39(4):591-607. Epub 2019 Sep 10 doi: 10.1016/j.cll.2019.07.007. PMID: 31668272

Therapy

Monaco MCG, Soldan SS, Su C, Clauze A, Cooper JF, Patel RJ, Lu F, Hughes RJ, Messick TE, Andrada FC, Ohayon J, Lieberman PM, Jacobson S
Neurol Neuroimmunol Neuroinflamm 2023 Sep;10(5) Epub 2023 Aug 10 doi: 10.1212/NXI.0000000000200149. PMID: 37562974Free PMC Article
Zografou C, Vakrakou AG, Stathopoulos P
Front Immunol 2021;12:686466. Epub 2021 Jun 17 doi: 10.3389/fimmu.2021.686466. PMID: 34220839Free PMC Article
Werner L, Dor C, Salamon N, Nagar M, Shouval DS
J Vis Exp 2021 Jan 12;(167) doi: 10.3791/61792. PMID: 33522509
Lyashchenko KP, Vordermeier HM, Waters WR
Vet Immunol Immunopathol 2020 Mar;221:110016. Epub 2020 Feb 2 doi: 10.1016/j.vetimm.2020.110016. PMID: 32050091
Ascough S, Paterson S, Chiu C
Front Immunol 2018;9:323. Epub 2018 Mar 2 doi: 10.3389/fimmu.2018.00323. PMID: 29552008Free PMC Article

Prognosis

Pulvirenti F, Mortari EP, Putotto C, Terreri S, Fernandez Salinas A, Cinicola BL, Cimini E, Di Napoli G, Sculco E, Milito C, Versacci P, Agrati C, Marino B, Carsetti R, Quinti I
J Allergy Clin Immunol Pract 2023 Jan;11(1):292-305.e2. Epub 2022 Oct 21 doi: 10.1016/j.jaip.2022.10.010. PMID: 36280136Free PMC Article
Lomakin YA, Zvyagin IV, Ovchinnikova LA, Kabilov MR, Staroverov DB, Mikelov A, Tupikin AE, Zakharova MY, Bykova NA, Mukhina VS, Favorov AV, Ivanova M, Simaniv T, Rubtsov YP, Chudakov DM, Zakharova MN, Illarioshkin SN, Belogurov AA Jr, Gabibov AG
Front Immunol 2022;13:803229. Epub 2022 Aug 16 doi: 10.3389/fimmu.2022.803229. PMID: 36052064Free PMC Article
Ricke-Hoch M, Stelling E, Lasswitz L, Gunesch AP, Kasten M, Zapatero-Belinchón FJ, Brogden G, Gerold G, Pietschmann T, Montiel V, Balligand JL, Facciotti F, Hirsch E, Gausepohl T, Elbahesh H, Rimmelzwaan GF, Höfer A, Kühnel MP, Jonigk D, Eigendorf J, Tegtbur U, Mink L, Scherr M, Illig T, Schambach A, Pfeffer TJ, Hilfiker A, Haverich A, Hilfiker-Kleiner D
PLoS One 2021;16(8):e0255335. Epub 2021 Aug 4 doi: 10.1371/journal.pone.0255335. PMID: 34347801Free PMC Article
Roussel L, Landekic M, Golizeh M, Gavino C, Zhong MC, Chen J, Faubert D, Blanchet-Cohen A, Dansereau L, Parent MA, Marin S, Luo J, Le C, Ford BR, Langelier M, King IL, Divangahi M, Foulkes WD, Veillette A, Vinh DC
J Exp Med 2018 Dec 3;215(12):3151-3164. doi: 10.1084/jem.20180668. PMID: 30498080Free PMC Article
Blanco-Quirós A, Solís-Sánchez P, Garrote-Adrados JA, Arranz-Sanz E
Allergol Immunopathol (Madr) 2006 Nov-Dec;34(6):263-75. doi: 10.1157/13095875. PMID: 17173844

Clinical prediction guides

Staniek J, Kalina T, Andrieux G, Boerries M, Janowska I, Fuentes M, Díez P, Bakardjieva M, Stancikova J, Raabe J, Neumann J, Schwenk S, Arpesella L, Stuchly J, Benes V, García Valiente R, Fernández García J, Carsetti R, Piano Mortari E, Catala A, de la Calle O, Sogkas G, Neven B, Rieux-Laucat F, Magerus A, Neth O, Olbrich P, Voll RE, Alsina L, Allende LM, Gonzalez-Granado LI, Böhler C, Thiel J, Venhoff N, Lorenzetti R, Warnatz K, Unger S, Seidl M, Mielenz D, Schneider P, Ehl S, Rensing-Ehl A, Smulski CR, Rizzi M
Sci Immunol 2024 Jan 12;9(91):eadj5948. doi: 10.1126/sciimmunol.adj5948. PMID: 38215192
Zografou C, Vakrakou AG, Stathopoulos P
Front Immunol 2021;12:686466. Epub 2021 Jun 17 doi: 10.3389/fimmu.2021.686466. PMID: 34220839Free PMC Article
Fernando SL, Jang HS, Li J
Immunol Lett 2021 Feb;230:21-26. Epub 2020 Dec 14 doi: 10.1016/j.imlet.2020.12.002. PMID: 33333111
Goswami M, Prince G, Biancotto A, Moir S, Kardava L, Santich BH, Cheung F, Kotliarov Y, Chen J, Shi R, Zhou H, Golding H, Manischewitz J, King L, Kunz LM, Noonan K, Borrello IM, Smith BD, Hourigan CS
J Transl Med 2017 Jul 10;15(1):155. doi: 10.1186/s12967-017-1252-2. PMID: 28693586Free PMC Article
Zouali M
Mol Immunol 2002 May;38(12-13):895-901. doi: 10.1016/s0161-5890(02)00016-0. PMID: 12009567

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