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MHC class II deficiency 2(MHC2D2)

MedGen UID:
347904
Concept ID:
C1859535
Disease or Syndrome
Synonym: Bare lymphocyte syndrome, type II, complementation group B
 
Gene (location): RFXANK (19p13.11)
 
Monarch Initiative: MONDO:0971013
OMIM®: 620815

Clinical features

From HPO
Failure to thrive
MedGen UID:
746019
Concept ID:
C2315100
Disease or Syndrome
Failure to thrive (FTT) refers to a child whose physical growth is substantially below the norm.
Hepatomegaly
MedGen UID:
42428
Concept ID:
C0019209
Finding
Abnormally increased size of the liver.
Chronic diarrhea
MedGen UID:
96036
Concept ID:
C0401151
Finding
The presence of chronic diarrhea, which is usually taken to mean diarrhea that has persisted for over 4 weeks.
Global developmental delay
MedGen UID:
107838
Concept ID:
C0557874
Finding
A delay in the achievement of motor or mental milestones in the domains of development of a child, including motor skills, speech and language, cognitive skills, and social and emotional skills. This term should only be used to describe children younger than five years of age.
Lower limb spasticity
MedGen UID:
220865
Concept ID:
C1271100
Finding
Spasticity (velocity-dependent increase in tonic stretch reflexes with increased muscle tone and hyperexcitable tendon reflexes) in the muscles of the lower limbs, hips, and pelvis.
Brain atrophy
MedGen UID:
1643639
Concept ID:
C4551584
Disease or Syndrome
Partial or complete wasting (loss) of brain tissue that was once present.
Hypotonia
MedGen UID:
10133
Concept ID:
C0026827
Finding
Hypotonia is an abnormally low muscle tone (the amount of tension or resistance to movement in a muscle). Even when relaxed, muscles have a continuous and passive partial contraction which provides some resistance to passive stretching. Hypotonia thus manifests as diminished resistance to passive stretching. Hypotonia is not the same as muscle weakness, although the two conditions can co-exist.
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 pneumonia
MedGen UID:
195802
Concept ID:
C0694550
Disease or Syndrome
An increased susceptibility to pneumonia as manifested by a history of recurrent episodes of pneumonia.
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.
Autoimmune hemolytic anemia
MedGen UID:
1918
Concept ID:
C0002880
Disease or Syndrome
An autoimmune form of hemolytic anemia.
Chronic mucocutaneous candidiasis
MedGen UID:
2426
Concept ID:
C0006845
Disease or Syndrome
Recurrent or persistent superficial Candida infections of the skin, mucous membranes, and nails.
Sclerosing cholangitis
MedGen UID:
3036
Concept ID:
C0008313
Disease or Syndrome
Cholangitis associated with evident ductal fibrosis that develops as a consequence of long-standing bile duct inflammatory, obstruction, or ischemic injury; it can be obliterative or nonobliterative.
Sepsis
MedGen UID:
48626
Concept ID:
C0036690
Disease or Syndrome
Systemic inflammatory response to infection.
Cytomegalovirus colitis
MedGen UID:
137956
Concept ID:
C0341335
Disease or Syndrome
A form of cytomegalovirus infection characterized by infection and inflammation of the colon.
Reduced antigen-specific T cell proliferation
MedGen UID:
1621145
Concept ID:
C4531148
Finding
Impaired proliferation and expansion of a T cell population following activation by an antigenic stimulus.
Cytoplasmic antineutrophil antibody positivity
MedGen UID:
1690999
Concept ID:
C5139210
Finding
The presence of autoantibodies in the serum that react against proteins predominantly expressed in cytoplasmic granules of neutrophils.
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

Korkmaz SB, Karaselek MA, Aytekin SE, Tokgoz H, Reisli I, Guner S, Keles S
Int J Immunogenet 2023 Aug;50(4):177-184. Epub 2023 Jun 12 doi: 10.1111/iji.12624. PMID: 37308802
Picard C, Fischer A
Immunol Allergy Clin North Am 2010 May;30(2):173-8. doi: 10.1016/j.iac.2010.01.001. PMID: 20493394

Recent clinical studies

Etiology

Gulec Koksal Z, Bilgic Eltan S, Topyildiz E, Sezer A, Keles S, Celebi Celik F, Ozhan Kont A, Gemici Karaaslan B, Sefer AP, Karali Z, Arik E, Ozek Yucel E, Akcal O, Karakurt LT, Yorgun Altunbas M, Yalcin K, Uygun V, Ozek G, Babayeva R, Aydogmus C, Ozcan D, Cavkaytar O, Keskin O, Kilic SS, Kiykim A, Arikoglu T, Genel F, Gulez N, Guner SN, Karaca NE, Reisli I, Kutukculer N, Altintas DU, Ozen A, Karakoc Aydiner E, Baris S
J Allergy Clin Immunol Pract 2024 Sep;12(9):2490-2502.e6. Epub 2024 Jul 10 doi: 10.1016/j.jaip.2024.06.046. PMID: 38996837
Hanna S, Etzioni A
J Allergy Clin Immunol 2014 Aug;134(2):269-75. Epub 2014 Jul 4 doi: 10.1016/j.jaci.2014.06.001. PMID: 25001848
Hanwell HE, Banwell B
Biochim Biophys Acta 2011 Feb;1812(2):202-12. Epub 2010 Jul 30 doi: 10.1016/j.bbadis.2010.07.017. PMID: 20674744
Fischer A
Immunodefic Rev 1992;3(2):83-100. PMID: 1554500
Yong K, Khwaja A
Blood Rev 1990 Dec;4(4):211-25. doi: 10.1016/0268-960x(90)90001-9. PMID: 1706206

Diagnosis

Gulec Koksal Z, Bilgic Eltan S, Topyildiz E, Sezer A, Keles S, Celebi Celik F, Ozhan Kont A, Gemici Karaaslan B, Sefer AP, Karali Z, Arik E, Ozek Yucel E, Akcal O, Karakurt LT, Yorgun Altunbas M, Yalcin K, Uygun V, Ozek G, Babayeva R, Aydogmus C, Ozcan D, Cavkaytar O, Keskin O, Kilic SS, Kiykim A, Arikoglu T, Genel F, Gulez N, Guner SN, Karaca NE, Reisli I, Kutukculer N, Altintas DU, Ozen A, Karakoc Aydiner E, Baris S
J Allergy Clin Immunol Pract 2024 Sep;12(9):2490-2502.e6. Epub 2024 Jul 10 doi: 10.1016/j.jaip.2024.06.046. PMID: 38996837
Alroqi FJ, Alhezam MA, Almojali AI, Barhoumi T, Althubaiti N, Alharbi Y, Al Balwi MA, Alrasheed A
J Clin Immunol 2024 Mar 1;44(3):73. doi: 10.1007/s10875-024-01674-0. PMID: 38424321Free PMC Article
Brogan P, Eleftheriou D
Pediatr Nephrol 2018 Feb;33(2):187-198. Epub 2017 Aug 7 doi: 10.1007/s00467-017-3597-4. PMID: 28785984Free PMC Article
Hanna S, Etzioni A
J Allergy Clin Immunol 2014 Aug;134(2):269-75. Epub 2014 Jul 4 doi: 10.1016/j.jaci.2014.06.001. PMID: 25001848
Fischer A
Immunodefic Rev 1992;3(2):83-100. PMID: 1554500

Therapy

Eguren-Santamaria I, Fernández de Piérola E, Camps G, Martín-Muñoz P, Campos M, Cuculescu D, Aguilera-Buenosvinos I, Rodríguez López I, Salido-Vallejo R, Alexandru R, De Andrea CE, Álvarez-Gigli L, Berraondo P, Melero I, Sanmamed MF
J Immunother Cancer 2024 Sep 6;12(9) doi: 10.1136/jitc-2023-008516. PMID: 39244214Free PMC Article
Hanwell HE, Banwell B
Biochim Biophys Acta 2011 Feb;1812(2):202-12. Epub 2010 Jul 30 doi: 10.1016/j.bbadis.2010.07.017. PMID: 20674744
Broides A, Shubinsky G, Parvari R, Grimbacher B, Somech R, Garty BZ, Levy J
Int J Immunogenet 2009 Aug;36(4):223-6. Epub 2009 May 19 doi: 10.1111/j.1744-313X.2009.00847.x. PMID: 19473215
Parvaneh N, Shahmahmoudi S, Tabatabai H, Zahraei M, Mousavi T, Esteghamati AR, Gooya MM, Mamishi S, Nategh R, Kew OM
J Clin Virol 2007 Jun;39(2):145-8. Epub 2007 May 16 doi: 10.1016/j.jcv.2007.04.002. PMID: 17509935
Fischer A
Immunodefic Rev 1992;3(2):83-100. PMID: 1554500

Prognosis

Gulec Koksal Z, Bilgic Eltan S, Topyildiz E, Sezer A, Keles S, Celebi Celik F, Ozhan Kont A, Gemici Karaaslan B, Sefer AP, Karali Z, Arik E, Ozek Yucel E, Akcal O, Karakurt LT, Yorgun Altunbas M, Yalcin K, Uygun V, Ozek G, Babayeva R, Aydogmus C, Ozcan D, Cavkaytar O, Keskin O, Kilic SS, Kiykim A, Arikoglu T, Genel F, Gulez N, Guner SN, Karaca NE, Reisli I, Kutukculer N, Altintas DU, Ozen A, Karakoc Aydiner E, Baris S
J Allergy Clin Immunol Pract 2024 Sep;12(9):2490-2502.e6. Epub 2024 Jul 10 doi: 10.1016/j.jaip.2024.06.046. PMID: 38996837
Smith CC, Olsen KS, Gentry KM, Sambade M, Beck W, Garness J, Entwistle S, Willis C, Vensko S, Woods A, Fini M, Carpenter B, Routh E, Kodysh J, O'Donnell T, Haber C, Heiss K, Stadler V, Garrison E, Sandor AM, Ting JPY, Weiss J, Krajewski K, Grant OC, Woods RJ, Heise M, Vincent BG, Rubinsteyn A
Genome Med 2021 Jun 14;13(1):101. doi: 10.1186/s13073-021-00910-1. PMID: 34127050Free PMC Article
Brogan P, Eleftheriou D
Pediatr Nephrol 2018 Feb;33(2):187-198. Epub 2017 Aug 7 doi: 10.1007/s00467-017-3597-4. PMID: 28785984Free PMC Article
Picard C, Fischer A
Immunol Allergy Clin North Am 2010 May;30(2):173-8. doi: 10.1016/j.iac.2010.01.001. PMID: 20493394
Parvaneh N, Shahmahmoudi S, Tabatabai H, Zahraei M, Mousavi T, Esteghamati AR, Gooya MM, Mamishi S, Nategh R, Kew OM
J Clin Virol 2007 Jun;39(2):145-8. Epub 2007 May 16 doi: 10.1016/j.jcv.2007.04.002. PMID: 17509935

Clinical prediction guides

Eguren-Santamaria I, Fernández de Piérola E, Camps G, Martín-Muñoz P, Campos M, Cuculescu D, Aguilera-Buenosvinos I, Rodríguez López I, Salido-Vallejo R, Alexandru R, De Andrea CE, Álvarez-Gigli L, Berraondo P, Melero I, Sanmamed MF
J Immunother Cancer 2024 Sep 6;12(9) doi: 10.1136/jitc-2023-008516. PMID: 39244214Free PMC Article
Gulec Koksal Z, Bilgic Eltan S, Topyildiz E, Sezer A, Keles S, Celebi Celik F, Ozhan Kont A, Gemici Karaaslan B, Sefer AP, Karali Z, Arik E, Ozek Yucel E, Akcal O, Karakurt LT, Yorgun Altunbas M, Yalcin K, Uygun V, Ozek G, Babayeva R, Aydogmus C, Ozcan D, Cavkaytar O, Keskin O, Kilic SS, Kiykim A, Arikoglu T, Genel F, Gulez N, Guner SN, Karaca NE, Reisli I, Kutukculer N, Altintas DU, Ozen A, Karakoc Aydiner E, Baris S
J Allergy Clin Immunol Pract 2024 Sep;12(9):2490-2502.e6. Epub 2024 Jul 10 doi: 10.1016/j.jaip.2024.06.046. PMID: 38996837
Brehm MA, Kenney LL, Wiles MV, Low BE, Tisch RM, Burzenski L, Mueller C, Greiner DL, Shultz LD
FASEB J 2019 Mar;33(3):3137-3151. Epub 2018 Nov 1 doi: 10.1096/fj.201800636R. PMID: 30383447Free PMC Article
Brogan P, Eleftheriou D
Pediatr Nephrol 2018 Feb;33(2):187-198. Epub 2017 Aug 7 doi: 10.1007/s00467-017-3597-4. PMID: 28785984Free PMC Article
Buonocore S, Van Meirvenne S, Demoor FX, Paulart F, Thielemans K, Goldman M, Flamand V
Transplantation 2002 Jan 15;73(1 Suppl):S27-30. doi: 10.1097/00007890-200201151-00010. PMID: 11810058

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