U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Immunodeficiency 120(IMD120)

MedGen UID:
1862745
Concept ID:
C5935622
Disease or Syndrome
Synonyms: IMD120; IMMUNODEFICIENCY 120
 
Gene (location): POLD1 (19q13.33)
 
Monarch Initiative: MONDO:0970994
OMIM®: 620836

Definition

Immunodeficiency-120 (IMD120) is an autosomal recessive combined immunodeficiency characterized by the onset of recurrent upper and lower respiratory tract infections in early childhood. Affected individuals may also develop persistent viral infections, particularly of the herpes family. Laboratory studies show lymphopenia with decreased CD4+ and CD8+ T cells and mildly decreased B cells, usually with hypogammaglobulinemia. Some patients may have additional systemic features, including hearing loss, speech delay, short stature, and mildly impaired intellectual development (Conde et al., 2019; et al., 2020). [from OMIM]

Clinical features

From HPO
Verrucae
MedGen UID:
777120
Concept ID:
C3665596
Finding
Warts, benign growths on the skin or mucous membranes that cause cosmetic problems as well as pain and discomfort. Warts most often occur on the hands, feet, and genital areas.
Short stature
MedGen UID:
87607
Concept ID:
C0349588
Finding
A height below that which is expected according to age and gender norms. Although there is no universally accepted definition of short stature, many refer to "short stature" as height more than 2 standard deviations below the mean for age and gender (or below the 3rd percentile for age and gender dependent norms).
Sensorineural hearing loss disorder
MedGen UID:
9164
Concept ID:
C0018784
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
Hearing impairment
MedGen UID:
235586
Concept ID:
C1384666
Disease or Syndrome
A decreased magnitude of the sensory perception of sound.
Intellectual disability, borderline
MedGen UID:
507499
Concept ID:
C0006009
Finding
Borderline intellectual disability is defined as an intelligence quotient (IQ) in the range of 70-85.
Delayed speech and language development
MedGen UID:
105318
Concept ID:
C0454644
Finding
A degree of language development that is significantly below the norm for a child of a specified age.
Microcephaly
MedGen UID:
1644158
Concept ID:
C4551563
Finding
Head circumference below 2 standard deviations below the mean for age and gender.
Bronchiectasis
MedGen UID:
14234
Concept ID:
C0006267
Disease or Syndrome
Persistent abnormal dilatation of the bronchi owing to localized and irreversible destruction and widening of the large airways.
Chronic bronchitis
MedGen UID:
3084
Concept ID:
C0008677
Disease or Syndrome
Chronic inflammation of the bronchi.
Recurrent upper respiratory tract infections
MedGen UID:
154380
Concept ID:
C0581381
Disease or Syndrome
An increased susceptibility to upper respiratory tract infections as manifested by a history of recurrent upper respiratory tract infections (running ears - otitis, sinusitis, pharyngitis, tonsillitis).
Recurrent lower respiratory tract infections
MedGen UID:
756211
Concept ID:
C3163798
Disease or Syndrome
An increased susceptibility to lower respiratory tract infections as manifested by a history of recurrent lower respiratory tract infections.
Decreased circulating IgA concentration
MedGen UID:
57934
Concept ID:
C0162538
Disease or Syndrome
Decreased levels of immunoglobulin A (IgA).
Decreased circulating total IgM
MedGen UID:
116095
Concept ID:
C0239989
Finding
An abnormally decreased level of immunoglobulin M (IgM) in blood.
Recurrent oral herpes
MedGen UID:
697662
Concept ID:
C1274321
Disease or Syndrome
Recurrent episodes of oral herpes, typically characterized by blisters or ulcers on the gums, lips and/or tongue caused by herpes virus.
B lymphocytopenia
MedGen UID:
340780
Concept ID:
C1855067
Finding
An abnormal decrease from the normal count of B cells.
Reduced natural killer cell count
MedGen UID:
383765
Concept ID:
C1855767
Finding
Less than normal number of natural killer cells, a type of lymphocyte in the innate immune system with an ability to mediate cytotoxicity and produce cytokines after the ligation of a germline-encoded activation receptor.
Recurrent shingles
MedGen UID:
816106
Concept ID:
C3809776
Disease or Syndrome
Repeated episodes of a localized, painful cutaneous eruption related to reactivation of varicella zoster virus (VZV) and characterized by a characteristic rash in one or two adjacent dermatomes.
Complete or near-complete absence of specific antibody response to tetanus vaccine
MedGen UID:
1687415
Concept ID:
C5139458
Finding
The inability to synthesize postvaccination antibodies against a tetanus antigen, as measured by antibody titer determination following vaccination.
Decreased proportion of naive CD4 T cells
MedGen UID:
1705154
Concept ID:
C5139539
Finding
An abnormally reduced proportion of naive CD4 T cells relative to the total number of T cells.
Decreased circulating IgG concentration
MedGen UID:
1720114
Concept ID:
C5234937
Finding
An abnormally decreased level of immunoglobulin G (IgG) in blood.
Decreased proportion of CD4-positive helper T cells
MedGen UID:
1719772
Concept ID:
C5235140
Finding
A decreased proportion of circulating CD4-positive helper T cells relative to total T cell count.

Professional guidelines

PubMed

FIGO Committee for Ethical Aspects of Human Reproduction and Women's Health
Int J Gynaecol Obstet 2013 Mar;120(3):310-1. Epub 2012 Nov 26 doi: 10.1016/j.ijgo.2012.10.007. PMID: 23195288
Janoff EN, Smith PD
Gastroenterology 2001 Feb;120(3):607-21. doi: 10.1053/gast.2001.22427. PMID: 11179239Free PMC Article
Miller KS, Miller JM Jr
Clin Obstet Gynecol 1996 Mar;39(1):120-42. doi: 10.1097/00003081-199603000-00012. PMID: 8635294

Recent clinical studies

Etiology

Shmakova A, Tsimailo I, Kozhevnikova Y, Gérard L, Boutboul D, Oksenhendler E, Tuaillon E, Rivault A, Germini D, Vassetzky Y, Beaumelle B
Int J Infect Dis 2024 May;142:106994. Epub 2024 Mar 4 doi: 10.1016/j.ijid.2024.106994. PMID: 38447753
Lian J, Chen X, Su X, Si M, Dai Z, Fu J, Yu F, Mi G, Liu Y
AIDS Care 2023 Apr;35(4):608-613. Epub 2022 Apr 8 doi: 10.1080/09540121.2022.2060493. PMID: 35392734
Qamar N, Fuleihan RL
Clin Rev Allergy Immunol 2014 Apr;46(2):120-30. doi: 10.1007/s12016-013-8378-7. PMID: 23797640
Stark D, Barratt JL, van Hal S, Marriott D, Harkness J, Ellis JT
Clin Microbiol Rev 2009 Oct;22(4):634-50. doi: 10.1128/CMR.00017-09. PMID: 19822892Free PMC Article
Wyatt CM, Klotman PE
Am J Med 2007 Jun;120(6):488-92. doi: 10.1016/j.amjmed.2007.01.025. PMID: 17524746

Diagnosis

Paschen-Wolff MM, Restar A, Gandhi AD, Serafino S, Sandfort T
AIDS Behav 2019 Apr;23(4):860-874. doi: 10.1007/s10461-019-02414-x. PMID: 30707329Free PMC Article
Welz T, Wyen C, Hensel M
Oncol Res Treat 2017;40(3):120-127. Epub 2017 Feb 24 doi: 10.1159/000458443. PMID: 28253501
Armstrong-James D, Meintjes G, Brown GD
Trends Microbiol 2014 Mar;22(3):120-7. Epub 2014 Feb 13 doi: 10.1016/j.tim.2014.01.001. PMID: 24530175
Qamar N, Fuleihan RL
Clin Rev Allergy Immunol 2014 Apr;46(2):120-30. doi: 10.1007/s12016-013-8378-7. PMID: 23797640
Azar AE, Ballas ZK
Am J Med 2007 Sep;120(9):764-8. doi: 10.1016/j.amjmed.2006.12.013. PMID: 17765042

Therapy

Margolis DM
Infect Dis Clin North Am 2024 Sep;38(3):487-497. Epub 2024 Jul 4 doi: 10.1016/j.idc.2024.06.001. PMID: 38969530Free PMC Article
Welz T, Wyen C, Hensel M
Oncol Res Treat 2017;40(3):120-127. Epub 2017 Feb 24 doi: 10.1159/000458443. PMID: 28253501
Bhutta Z, Yackobovitch-Gavan M
World Rev Nutr Diet 2016;114:120-7. Epub 2016 Feb 23 doi: 10.1159/000441823. PMID: 26914906
Kim JM, Han SH
Immunotherapy 2011 Feb;3(2):247-67. doi: 10.2217/imt.10.91. PMID: 21322762
Drake TE, Maibach HI
Postgrad Med 1973 Mar;53(3):120-5. doi: 10.1080/00325481.1973.11713396. PMID: 4570710

Prognosis

Sittithumcharee G, Kariya R, Kasemsuk T, Saeeng R, Okada S
Phytother Res 2021 Dec;35(12):7018-7026. Epub 2021 Nov 15 doi: 10.1002/ptr.7322. PMID: 34779075
Antonini M, Pontes PS, Melo ES, de Souza Alves R, Gir E, Sorensen W, Reis RK
BMC Public Health 2021 Oct 13;21(1):1849. doi: 10.1186/s12889-021-11835-0. PMID: 34645401Free PMC Article
Foreman KJ, Marquez N, Dolgert A, Fukutaki K, Fullman N, McGaughey M, Pletcher MA, Smith AE, Tang K, Yuan CW, Brown JC, Friedman J, He J, Heuton KR, Holmberg M, Patel DJ, Reidy P, Carter A, Cercy K, Chapin A, Douwes-Schultz D, Frank T, Goettsch F, Liu PY, Nandakumar V, Reitsma MB, Reuter V, Sadat N, Sorensen RJD, Srinivasan V, Updike RL, York H, Lopez AD, Lozano R, Lim SS, Mokdad AH, Vollset SE, Murray CJL
Lancet 2018 Nov 10;392(10159):2052-2090. Epub 2018 Oct 16 doi: 10.1016/S0140-6736(18)31694-5. PMID: 30340847Free PMC Article
Lanaspa M, Annamalay AA, LeSouëf P, Bassat Q
Expert Rev Anti Infect Ther 2014 Jan;12(1):31-47. doi: 10.1586/14787210.2014.866517. PMID: 24410617Free PMC Article
Szabolcs P, Cavazzana-Calvo M, Fischer A, Veys P
Pediatr Clin North Am 2010 Feb;57(1):207-37. doi: 10.1016/j.pcl.2009.12.004. PMID: 20307719

Clinical prediction guides

Dash PK, Chen C, Kaminski R, Su H, Mancuso P, Sillman B, Zhang C, Liao S, Sravanam S, Liu H, Waight E, Guo L, Mathews S, Sariyer R, Mosley RL, Poluektova LY, Caocci M, Amini S, Gorantla S, Burdo TH, Edagwa B, Gendelman HE, Khalili K
Proc Natl Acad Sci U S A 2023 May 9;120(19):e2217887120. Epub 2023 May 1 doi: 10.1073/pnas.2217887120. PMID: 37126704Free PMC Article
Antonini M, Pontes PS, Melo ES, de Souza Alves R, Gir E, Sorensen W, Reis RK
BMC Public Health 2021 Oct 13;21(1):1849. doi: 10.1186/s12889-021-11835-0. PMID: 34645401Free PMC Article
Hanne J, Göttfert F, Schimer J, Anders-Össwein M, Konvalinka J, Engelhardt J, Müller B, Hell SW, Kräusslich HG
ACS Nano 2016 Sep 27;10(9):8215-22. Epub 2016 Aug 16 doi: 10.1021/acsnano.6b03850. PMID: 27517329
Hodge WG, Boivin JF, Shapiro SH, Shah KC, Dionne MA
Can J Ophthalmol 2005 Dec;40(6):701-10. doi: 10.1016/s0008-4182(05)80086-9. PMID: 16518896
Schiødt M, Pindborg JJ
Int J Oral Maxillofac Surg 1987 Feb;16(1):1-14. doi: 10.1016/s0901-5027(87)80025-5. PMID: 3104486

Recent systematic reviews

Wilson L, Gracie L, Kidy F, Thomas GN, Nirantharakumar K, Greenfield S, Manaseki-Holland S, Ward DJ, Gooden TE
BMC Infect Dis 2023 Feb 24;23(1):120. doi: 10.1186/s12879-023-08092-4. PMID: 36829123Free PMC Article
Barron K, Omiunu A, Celidonio J, Cruz-Mullane A, Din-Lovinescu C, Chemas-Velez MM, Baredes S, Eloy JA, Fang CH
Otolaryngol Head Neck Surg 2023 Mar;168(3):269-281. Epub 2023 Jan 24 doi: 10.1177/01945998221105059. PMID: 35671149
Sadeq A, Elnour AA, Farah FH, Ramadan A, Baraka MA, Don J, Amoodi AA, Sam KG, Mazrouei NA, Alkaabi M
Curr Rev Clin Exp Pharmacol 2023;18(2):120-147. doi: 10.2174/2772432817666220531115314. PMID: 35642121
Kay AW, Ness T, Verkuijl SE, Viney K, Brands A, Masini T, González Fernández L, Eisenhut M, Detjen AK, Mandalakas AM, Steingart KR, Takwoingi Y
Cochrane Database Syst Rev 2022 Sep 6;9(9):CD013359. doi: 10.1002/14651858.CD013359.pub3. PMID: 36065889Free PMC Article
Scott-Sheldon LAJ, Balletto BL, Donahue ML, Feulner MM, Cruess DG, Salmoirago-Blotcher E, Wing RR, Carey MP
AIDS Behav 2019 Jan;23(1):60-75. doi: 10.1007/s10461-018-2236-9. PMID: 30054765Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...