U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Tetrasomy 21

MedGen UID:
1640242
Concept ID:
C4707057
Disease or Syndrome
Synonyms: Isochromosome 21; tetrasomy 21; tetrasomy type 21
SNOMED CT: Tetrasomy 21 (764690001); Isochromosome 21 (764690001)
 
Monarch Initiative: MONDO:0019864
Orphanet: ORPHA96055

Definition

An extremely rare autosomal anomaly resulting from the presence of 4 copies of chromosome 21. The disease is characterised by features of trisomy 21 including developmental delay/intellectual disability, muscular hypotonia, short neck with redundant skin, brachycephaly, microcephaly, flat face, epicanthus, upslanted palpebral fissures, small ears, protruding tongue, single transverse palmar crease, brachydactyly, hypoplastic iliac wings, together with additional features such as prematurity, intrauterine growth retardation, high and broad forehead, hypertelorism. Haematological malignancies are also associated and may occur earlier than in trisomy 21. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTetrasomy 21

Professional guidelines

PubMed

Hu H, Huang Y, Hou R, Xu H, Liu Y, Liao X, Xu J, Jiang L, Wang D
BMC Med Genomics 2023 Apr 3;16(1):69. doi: 10.1186/s12920-023-01493-z. PMID: 37013593Free PMC Article
Lapierre JM, Cacheux V, Collot N, Da Silva F, Hervy N, Rivet D, Romana S, Wiss J, Benzaken B, Aurias A, Tachdjian G
Ann Genet 1998;41(3):133-40. PMID: 9833066
Yu LC, Moore DH 2nd, Magrane G, Cronin J, Pinkel D, Lebo RV, Gray JW
Cytometry 1997 Jul 1;28(3):191-7. PMID: 9222103

Recent clinical studies

Etiology

Hasle H, Kline RM, Kjeldsen E, Nik-Abdul-Rashid NF, Bhojwani D, Verboon JM, DiTroia SP, Chao KR, Raaschou-Jensen K, Palle J, Zwaan CM, Nyvold CG, Sankaran VG, Cantor AB
Blood 2022 May 26;139(21):3159-3165. doi: 10.1182/blood.2021011463. PMID: 34758059Free PMC Article
Li FX, Xie MJ, Qu SF, He D, Wu L, Liang ZK, Wu YS, Yang F, Yang XX
Mol Med Rep 2020 Aug;22(2):1269-1276. Epub 2020 Jun 3 doi: 10.3892/mmr.2020.11208. PMID: 32626971Free PMC Article
Gatinois V, Bigi N, Mousty E, Chiesa J, Musizzano Y, Schneider A, Lefort G, Pinson L, Gaillard JB, Ragon C, Perez MJ, Tournaire M, Blanchet P, Corsini C, Haquet E, Callier P, Geneviève D, Pellestor F, Puechberty J
Mol Genet Genomic Med 2019 Nov;7(11):e00895. Epub 2019 Sep 7 doi: 10.1002/mgg3.895. PMID: 31493343Free PMC Article
Kondoh T, Kobayashi D, Tsuji N, Kuribayashi K, Watanabe N
Exp Hematol 2009 Jul;37(7):824-30. Epub 2009 May 3 doi: 10.1016/j.exphem.2009.04.010. PMID: 19409952
Sandoval C, Pine SR, Guo Q, Sastry S, Stewart J, Kronn D, Jayabose S
Pediatr Blood Cancer 2005 Jan;44(1):85-91. doi: 10.1002/pbc.20161. PMID: 15390279

Diagnosis

Li FX, Xie MJ, Qu SF, He D, Wu L, Liang ZK, Wu YS, Yang F, Yang XX
Mol Med Rep 2020 Aug;22(2):1269-1276. Epub 2020 Jun 3 doi: 10.3892/mmr.2020.11208. PMID: 32626971Free PMC Article
Gatinois V, Bigi N, Mousty E, Chiesa J, Musizzano Y, Schneider A, Lefort G, Pinson L, Gaillard JB, Ragon C, Perez MJ, Tournaire M, Blanchet P, Corsini C, Haquet E, Callier P, Geneviève D, Pellestor F, Puechberty J
Mol Genet Genomic Med 2019 Nov;7(11):e00895. Epub 2019 Sep 7 doi: 10.1002/mgg3.895. PMID: 31493343Free PMC Article
Sandoval C, Pine SR, Guo Q, Sastry S, Stewart J, Kronn D, Jayabose S
Pediatr Blood Cancer 2005 Jan;44(1):85-91. doi: 10.1002/pbc.20161. PMID: 15390279
Lopes V, Mak E, Wyatt PR
Prenat Diagn 1985 May-Jun;5(3):233-5. doi: 10.1002/pd.1970050312. PMID: 3161017
Gilgenkrantz S, Droulle P, Schweitzer M, Foliguet B, Chadefaux B, Lombard M, Chery M, Prieur M
Clin Genet 1985 Dec;28(6):495-502. doi: 10.1111/j.1399-0004.1985.tb00416.x. PMID: 2934184

Therapy

Kondoh T, Kobayashi D, Tsuji N, Kuribayashi K, Watanabe N
Exp Hematol 2009 Jul;37(7):824-30. Epub 2009 May 3 doi: 10.1016/j.exphem.2009.04.010. PMID: 19409952
Sandoval C, Pine SR, Guo Q, Sastry S, Stewart J, Kronn D, Jayabose S
Pediatr Blood Cancer 2005 Jan;44(1):85-91. doi: 10.1002/pbc.20161. PMID: 15390279
Minelli A, Morerio C, Maserati E, Olivieri C, Panarello C, Bonvini L, Leszl A, Rosanda C, Lanino E, Danesino C, Pasquali F
Leukemia 2001 Jun;15(6):971-5. doi: 10.1038/sj.leu.2402123. PMID: 11417485

Prognosis

Rothman R, Trakhtenbrot L, Bielorai B, Izraeli S, Ishoev G, Amariglio N, Rechavi G, Toren A
Br J Haematol 2005 May;129(4):491-8. doi: 10.1111/j.1365-2141.2005.05479.x. PMID: 15877731
Sandoval C, Pine SR, Guo Q, Sastry S, Stewart J, Kronn D, Jayabose S
Pediatr Blood Cancer 2005 Jan;44(1):85-91. doi: 10.1002/pbc.20161. PMID: 15390279
Paulsson K, Panagopoulos I, Knuutila S, Jee KJ, Garwicz S, Fioretos T, Mitelman F, Johansson B
Blood 2003 Oct 15;102(8):3010-5. Epub 2003 Jun 26 doi: 10.1182/blood-2003-05-1444. PMID: 12829594
Minelli A, Morerio C, Maserati E, Olivieri C, Panarello C, Bonvini L, Leszl A, Rosanda C, Lanino E, Danesino C, Pasquali F
Leukemia 2001 Jun;15(6):971-5. doi: 10.1038/sj.leu.2402123. PMID: 11417485
Soler A, Margarit E, Carrió A, Costa D, Queralt R, Ballesta F
J Med Genet 1999 Apr;36(4):333-4. PMID: 10227405Free PMC Article

Clinical prediction guides

Takano T, Nakabayashi K, Ota H, Arai Y, Kamura H, Hata K
Eur J Med Genet 2020 Apr;63(4):103824. Epub 2019 Dec 9 doi: 10.1016/j.ejmg.2019.103824. PMID: 31830537
Gatinois V, Bigi N, Mousty E, Chiesa J, Musizzano Y, Schneider A, Lefort G, Pinson L, Gaillard JB, Ragon C, Perez MJ, Tournaire M, Blanchet P, Corsini C, Haquet E, Callier P, Geneviève D, Pellestor F, Puechberty J
Mol Genet Genomic Med 2019 Nov;7(11):e00895. Epub 2019 Sep 7 doi: 10.1002/mgg3.895. PMID: 31493343Free PMC Article
Sandoval C, Pine SR, Guo Q, Sastry S, Stewart J, Kronn D, Jayabose S
Pediatr Blood Cancer 2005 Jan;44(1):85-91. doi: 10.1002/pbc.20161. PMID: 15390279
Ohsaka A, Hisa T, Watanabe N, Kojima H, Nagasawa T
Cancer Genet Cytogenet 2002 Apr 1;134(1):60-4. doi: 10.1016/s0165-4608(01)00604-5. PMID: 11996798
Zaccaria A, Rosti G, Testoni N, Mazza P, Cantore M, Tura S
Cancer Genet Cytogenet 1983 Jul;9(3):217-26. doi: 10.1016/0165-4608(83)90004-3. PMID: 6861114

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.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...