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t(3;21)(q26;q22)

MedGen UID:
308377
•Concept ID:
C1515763
•
Cell or Molecular Dysfunction
Synonym: t(3;21)(q26.2;q22)

Definition

A cytogenetic abnormality that refers to the translocation of the long arm (q26) of chromosome 3 and the long arm (q22) of chromosome 21. It is associated with RUNX1/MECOM fusions, chronic myelogenous leukemia in blast crisis (BC-CML), myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVt(3;21)(q26;q22)

Recent clinical studies

Etiology

Cytogenetic and molecular genetic evolution of chronic myeloid leukemia.
Johansson B, Fioretos T, Mitelman F
Acta Haematol 2002;107(2):76-94. doi: 10.1159/000046636. PMID: 11919388
Abnormalities of 3q21 and 3q26 in myeloid malignancy: a United Kingdom Cancer Cytogenetic Group study.
Secker-Walker LM, Mehta A, Bain B
Br J Haematol 1995 Oct;91(2):490-501. doi: 10.1111/j.1365-2141.1995.tb05329.x. PMID: 8547101
The cytogenetic scenario of chronic myeloid leukemia.
Mitelman F
Leuk Lymphoma 1993;11 Suppl 1:11-5. doi: 10.3109/10428199309047856. PMID: 8251885

Diagnosis

A primary pediatric acute myelomonocytic leukemia with t(3;21)(q26;q22): A case report.
Duan JX, Liu F, Chang L, Che GL, Yang QX, Teng J, Jian H, Liu XJ, Lai SY
Medicine (Baltimore) 2023 Oct 27;102(43):e35721. doi: 10.1097/MD.0000000000035721. PMID: 37904382Free PMC Article
Acute promyelocytic leukemia relapsing as secondary acute myelogenous leukemia with translocation t(3;21)(q26;q22) and RUNX1-MDS1-EVI1 fusion transcript.
Park TS, Choi JR, Yoon SH, Song J, Kim J, Kim SJ, Kwon O, Min YH
Cancer Genet Cytogenet 2008 Dec;187(2):61-73. doi: 10.1016/j.cancergencyto.2008.06.015. PMID: 19027486
Acute myeloid leukemia (AML) with t(8;21)(q22;q22) relapsing as AML with t(3;21)(q26;q22).
Bacher U, Schnittger S, Kern W, Trenn G, Weisser M, Haferlach T, Schoch C
Cancer Genet Cytogenet 2006 Jul 15;168(2):172-4. doi: 10.1016/j.cancergencyto.2006.02.011. PMID: 16843110
Cytogenetic and molecular genetic evolution of chronic myeloid leukemia.
Johansson B, Fioretos T, Mitelman F
Acta Haematol 2002;107(2):76-94. doi: 10.1159/000046636. PMID: 11919388
t(3;21)(q26;q22): a recurring chromosomal abnormality in therapy-related myelodysplastic syndrome and acute myeloid leukemia.
Rubin CM, Larson RA, Anastasi J, Winter JN, Thangavelu M, Vardiman JW, Rowley JD, Le Beau MM
Blood 1990 Dec 15;76(12):2594-8. PMID: 2265251

Therapy

Acute promyelocytic leukemia relapsing as secondary acute myelogenous leukemia with translocation t(3;21)(q26;q22) and RUNX1-MDS1-EVI1 fusion transcript.
Park TS, Choi JR, Yoon SH, Song J, Kim J, Kim SJ, Kwon O, Min YH
Cancer Genet Cytogenet 2008 Dec;187(2):61-73. doi: 10.1016/j.cancergencyto.2008.06.015. PMID: 19027486
t(3;21)(q26;q22) in myeloid leukemia: an aggressive syndrome of blast transformation associated with hydroxyurea or antimetabolite therapy.
Yin CC, Cortes J, Barkoh B, Hayes K, Kantarjian H, Jones D
Cancer 2006 Apr 15;106(8):1730-8. doi: 10.1002/cncr.21797. PMID: 16532439
Cytogenetic and molecular genetic evolution of chronic myeloid leukemia.
Johansson B, Fioretos T, Mitelman F
Acta Haematol 2002;107(2):76-94. doi: 10.1159/000046636. PMID: 11919388
Remission of Philadelphia positive chronic myelogenous leukemia associated with t(3;21) after bone marrow transplantation.
Uriarte MR, Mori MA, de Bellis R, Cardoso H
Cancer Genet Cytogenet 1993 Jul 15;68(2):122-5. doi: 10.1016/0165-4608(93)90007-9. PMID: 8353803

Prognosis

A primary pediatric acute myelomonocytic leukemia with t(3;21)(q26;q22): A case report.
Duan JX, Liu F, Chang L, Che GL, Yang QX, Teng J, Jian H, Liu XJ, Lai SY
Medicine (Baltimore) 2023 Oct 27;102(43):e35721. doi: 10.1097/MD.0000000000035721. PMID: 37904382Free PMC Article
Cytogenetic and molecular genetic evolution of chronic myeloid leukemia.
Johansson B, Fioretos T, Mitelman F
Acta Haematol 2002;107(2):76-94. doi: 10.1159/000046636. PMID: 11919388
AML1 fusion transcripts in t(3;21) positive leukemia: evidence of molecular heterogeneity and usage of splicing sites frequently involved in the generation of normal AML1 transcripts.
Sacchi N, Nisson PE, Watkins PC, Faustinella F, Wijsman J, Hagemeijer A
Genes Chromosomes Cancer 1994 Dec;11(4):226-36. doi: 10.1002/gcc.2870110405. PMID: 7533526
The cytogenetic scenario of chronic myeloid leukemia.
Mitelman F
Leuk Lymphoma 1993;11 Suppl 1:11-5. doi: 10.3109/10428199309047856. PMID: 8251885
Translocation (3;21) characterizes crises in myeloid stem cell disorders.
Chen Z, Morgan R, Baer MR, Ligorsky R, Sandberg AA
Cancer Genet Cytogenet 1991 Dec;57(2):153-9. doi: 10.1016/0165-4608(91)90146-l. PMID: 1756492

Clinical prediction guides

Role of the RUNX1-EVI1 fusion gene in leukemogenesis.
Maki K, Yamagata T, Mitani K
Cancer Sci 2008 Oct;99(10):1878-83. doi: 10.1111/j.1349-7006.2008.00956.x. PMID: 19016745Free PMC Article
t(3;21)(q26;q22) in myeloid leukemia: an aggressive syndrome of blast transformation associated with hydroxyurea or antimetabolite therapy.
Yin CC, Cortes J, Barkoh B, Hayes K, Kantarjian H, Jones D
Cancer 2006 Apr 15;106(8):1730-8. doi: 10.1002/cncr.21797. PMID: 16532439
The EVI1 gene in myeloid leukemia.
Nucifora G
Leukemia 1997 Dec;11(12):2022-31. doi: 10.1038/sj.leu.2400880. PMID: 9447815
AML1 fusion transcripts in t(3;21) positive leukemia: evidence of molecular heterogeneity and usage of splicing sites frequently involved in the generation of normal AML1 transcripts.
Sacchi N, Nisson PE, Watkins PC, Faustinella F, Wijsman J, Hagemeijer A
Genes Chromosomes Cancer 1994 Dec;11(4):226-36. doi: 10.1002/gcc.2870110405. PMID: 7533526
t(3;21)(q26;q22): a recurring chromosomal abnormality in therapy-related myelodysplastic syndrome and acute myeloid leukemia.
Rubin CM, Larson RA, Anastasi J, Winter JN, Thangavelu M, Vardiman JW, Rowley JD, Le Beau MM
Blood 1990 Dec 15;76(12):2594-8. PMID: 2265251

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