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Leukemia, acute, X-linked

MedGen UID:
502257
•Concept ID:
C3501854
•
Neoplastic Process
Synonym: Leukemia, Acute, X-Linked
 
Monarch Initiative: MONDO:0700060
OMIM®: 308960

Definition

X-linked form of acute leukemia [from MONDO]

Clinical features

From HPO
Acute leukemia
MedGen UID:
43225
•Concept ID:
C0085669
•
Neoplastic Process
A clonal (malignant) hematopoietic disorder with an acute onset, affecting the bone marrow and the peripheral blood. The malignant cells show minimal differentiation and are called blasts, either myeloid blasts (myeloblasts) or lymphoid blasts (lymphoblasts).
See: Feature record | Search on this feature

Professional guidelines

PubMed

Pediatric leukemia susceptibility disorders: manifestations and management.
McReynolds LJ, Savage SA
Hematology Am Soc Hematol Educ Program 2017 Dec 8;2017(1):242-250. doi: 10.1182/asheducation-2017.1.242. PMID: 29222262Free PMC Article
Gene transfer into hematopoietic stem cells as treatment for primary immunodeficiency diseases.
Candotti F
Int J Hematol 2014 Apr;99(4):383-92. Epub 2014 Feb 1 doi: 10.1007/s12185-014-1524-z. PMID: 24488786
Congenital neutropenia: diagnosis, molecular bases and patient management.
Donadieu J, Fenneteau O, Beaupain B, Mahlaoui N, Chantelot CB
Orphanet J Rare Dis 2011 May 19;6:26. doi: 10.1186/1750-1172-6-26. PMID: 21595885Free PMC Article

Recent clinical studies

Etiology

Identification of USP9X as a leukemia susceptibility gene.
Sisoudiya SD, Mishra P, Li H, Schraw JM, Scheurer ME, Salvi S, Doddapaneni H, Muzny D, Mitchell D, Taylor O, Sabo A, Lupo PJ, Plon SE
Blood Adv 2023 Aug 22;7(16):4563-4575. doi: 10.1182/bloodadvances.2023009814. PMID: 37289514Free PMC Article
HMGA2 regulates acute myeloid leukemia progression and sensitivity to daunorubicin via Wnt/β-catenin signaling.
Yang S, Gu Y, Wang G, Hu Q, Chen S, Wang Y, Zhao M
Int J Mol Med 2019 Aug;44(2):427-436. Epub 2019 Jun 5 doi: 10.3892/ijmm.2019.4229. PMID: 31173171Free PMC Article
Congenital neutropenia: diagnosis, molecular bases and patient management.
Donadieu J, Fenneteau O, Beaupain B, Mahlaoui N, Chantelot CB
Orphanet J Rare Dis 2011 May 19;6:26. doi: 10.1186/1750-1172-6-26. PMID: 21595885Free PMC Article
Fanconi anemia.
Green AM, Kupfer GM
Hematol Oncol Clin North Am 2009 Apr;23(2):193-214. doi: 10.1016/j.hoc.2009.01.008. PMID: 19327579Free PMC Article
Bone marrow failure syndromes in children.
Alter BP
Pediatr Clin North Am 2002 Oct;49(5):973-88. doi: 10.1016/s0031-3955(02)00031-7. PMID: 12430621

Diagnosis

VEXAS syndrome: A review of bone marrow aspirate and biopsies reporting myeloid and erythroid precursor vacuolation.
Cherniawsky H, Friedmann J, Nicolson H, Dehghan N, Stubbins RJ, Foltz LM, Leitch HA, Sreenivasan GM, Ambler KLS, Nevill TJ, McGinnis E, Wilson L, Beck DB, Chen LYC, Marcon KM
Eur J Haematol 2023 Jun;110(6):633-638. Epub 2023 Feb 22 doi: 10.1111/ejh.13944. PMID: 36788756
HMGA2 regulates acute myeloid leukemia progression and sensitivity to daunorubicin via Wnt/β-catenin signaling.
Yang S, Gu Y, Wang G, Hu Q, Chen S, Wang Y, Zhao M
Int J Mol Med 2019 Aug;44(2):427-436. Epub 2019 Jun 5 doi: 10.3892/ijmm.2019.4229. PMID: 31173171Free PMC Article
Congenital neutropenia: diagnosis, molecular bases and patient management.
Donadieu J, Fenneteau O, Beaupain B, Mahlaoui N, Chantelot CB
Orphanet J Rare Dis 2011 May 19;6:26. doi: 10.1186/1750-1172-6-26. PMID: 21595885Free PMC Article
Fanconi anemia.
Green AM, Kupfer GM
Hematol Oncol Clin North Am 2009 Apr;23(2):193-214. doi: 10.1016/j.hoc.2009.01.008. PMID: 19327579Free PMC Article
Bone marrow failure syndromes in children.
Alter BP
Pediatr Clin North Am 2002 Oct;49(5):973-88. doi: 10.1016/s0031-3955(02)00031-7. PMID: 12430621

Therapy

Combined inhibition of XIAP and autophagy induces apoptosis and differentiation in acute myeloid leukaemia.
Huang Z, Zhou J, Jiang Y, Han Y, Wang X, Li F, Jiang S, Yu K, Zhang S
J Cell Mol Med 2023 Jun;27(12):1682-1696. Epub 2023 May 8 doi: 10.1111/jcmm.17765. PMID: 37154878Free PMC Article
Targeting apoptosis in acute myeloid leukaemia.
Cassier PA, Castets M, Belhabri A, Vey N
Br J Cancer 2017 Oct 10;117(8):1089-1098. Epub 2017 Aug 24 doi: 10.1038/bjc.2017.281. PMID: 29017180Free PMC Article
Gene transfer into hematopoietic stem cells as treatment for primary immunodeficiency diseases.
Candotti F
Int J Hematol 2014 Apr;99(4):383-92. Epub 2014 Feb 1 doi: 10.1007/s12185-014-1524-z. PMID: 24488786
Congenital neutropenia: diagnosis, molecular bases and patient management.
Donadieu J, Fenneteau O, Beaupain B, Mahlaoui N, Chantelot CB
Orphanet J Rare Dis 2011 May 19;6:26. doi: 10.1186/1750-1172-6-26. PMID: 21595885Free PMC Article
Fanconi anemia.
Green AM, Kupfer GM
Hematol Oncol Clin North Am 2009 Apr;23(2):193-214. doi: 10.1016/j.hoc.2009.01.008. PMID: 19327579Free PMC Article

Prognosis

ZFX-mediated upregulation of CEBPA-AS1 contributes to acute myeloid leukemia progression through miR-24-3p/CTBP2 axis.
Wang C, Song CM, Liu S, Chen LM, Xue SF, Huang SH, Lin H, Liu GH
Cell Biol Toxicol 2023 Dec;39(6):2631-2645. Epub 2023 Jan 30 doi: 10.1007/s10565-023-09792-y. PMID: 36715854
The role of FOXP3 rs3761548 and rs2294021 polymorphisms in pediatrics acute lymphoblastic leukemia: association with risk and response to therapy.
Ghasemi Z, Kalantar K, Amirghofran Z
Mol Biol Rep 2021 Feb;48(2):1139-1150. Epub 2021 Jan 30 doi: 10.1007/s11033-021-06154-x. PMID: 33517519
Prognostic value of inhibitor of apoptosis protein family expression in patients with acute myeloid leukemia.
Pluta A, Wierzbowska A, Cebula-Obrzut B, Pluta P, Stępka K, Szmigielska-Kapłon A, Grzybowska-Izydorczyk O, Czemerska M, Smolewski P, Wrzesien-Kus A, Robak T
Leuk Lymphoma 2015;56(9):2529-35. Epub 2015 Feb 20 doi: 10.3109/10428194.2014.1003052. PMID: 25549803
Clonal diseases of large granular lymphocytes.
Loughran TP Jr
Blood 1993 Jul 1;82(1):1-14. PMID: 8324214
Acute myeloid leukemia: analysis of ras gene mutations and clonality defined by polymorphic X-linked loci.
Bartram CR, Ludwig WD, Hiddemann W, Lyons J, Buschle M, Ritter J, Harbott J, Fröhlich A, Janssen JW
Leukemia 1989 Apr;3(4):247-56. PMID: 2564452

Clinical prediction guides

The role of FOXP3 rs3761548 and rs2294021 polymorphisms in pediatrics acute lymphoblastic leukemia: association with risk and response to therapy.
Ghasemi Z, Kalantar K, Amirghofran Z
Mol Biol Rep 2021 Feb;48(2):1139-1150. Epub 2021 Jan 30 doi: 10.1007/s11033-021-06154-x. PMID: 33517519
Synergism between NF-kappa B inhibitor, celastrol, and XIAP inhibitor, embelin, in an acute myeloid leukemia cell line, HL-60.
Pazhang Y, Jaliani HZ, Imani M, Dariushnejad H
J Cancer Res Ther 2016 Jan-Mar;12(1):155-60. doi: 10.4103/0973-1482.150407. PMID: 27072230
Adamantyl-substituted retinoid-related molecules induce apoptosis in human acute myelogenous leukemia cells.
Farhana L, Dawson MI, Xia Z, Aboukameel A, Xu L, Liu G, Das JK, Hatfield J, Levi E, Mohammad R, Fontana JA
Mol Cancer Ther 2010 Nov;9(11):2903-13. Epub 2010 Nov 9 doi: 10.1158/1535-7163.MCT-10-0546. PMID: 21062916Free PMC Article
Annexinopathies.
Hayes MJ, Longbottom RE, Evans MA, Moss SE
Subcell Biochem 2007;45:1-28. doi: 10.1007/978-1-4020-6191-2_1. PMID: 18193632
Bone marrow failure syndromes in children.
Alter BP
Pediatr Clin North Am 2002 Oct;49(5):973-88. doi: 10.1016/s0031-3955(02)00031-7. PMID: 12430621

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