precursor T-lymphoblastic leukemia/lymphoma
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Epidemiology
- most common in young men
Genetics
- t(11;14)(p15;q11) involving T-cell receptor delta chain & rhombotin-1 genes
- t(11;14)(p13;q11) involving T-cell receptor delta chain & rhombotin-2 genes
- t(1;14)(p32;q11) involving T-cell receptor alpha chain & TAL1 genes
- t(3;11)(q12.2;p15.4) involving LNP1 with NUP98
- t(4;16)(q26;p13) involving IL2 with TNFRSF17
- t(7;9)(q34;q32) involving TAL2 with TCRB
- t(7;19)(q35;p13) involving LYL1 with TCRB
- t(10;14)(q24;q11) involving TLX1 (HOX11) with TCRD
- t(14;21)(q11.2;q22) involving OLIG2 with TCRA
- STIL/TAL1 internal deletion, chromosomal deletion 1p32
- notch-1 truncation
Clinical manifestations
- most frequently presents with mediastinal mass
Laboratory
- serum LDH has prognostic value
- lumbar puncture with CSF analysis
- chromosome region 1p32 deletion STIL,TAL1 fusion portends good prognosis in children
Management
- chemotherapy
- central nervous system prophylaxis
- donor-derived anti-CD7 CAR T-cell therapy
- complete response in 90% of relapsed or refractory cases in small study[3]
More general terms
More specific terms
References
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 11, American College of Physicians, Philadelphia 1998
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 519
- ↑ 3.0 3.1 Bankhead C Anti-CD7 CAR-T Therapy Yields High Response Rate in Acute Leukemia. Complete responses in 18 of 20 patients with relapsed/refractory T-cell ALL. MedPage Today August 4, 2021 https://www.medpagetoday.com/hematologyoncology/leukemia/93902
Pan J, Tan Y, Wang G et al Donor-derived CD7 chimeric antigen receptor T cells for T-cell acute lymphoblastic leukemia: First-in-human, phase I trial. J Clin Oncol. 2021. Jul 29 PMID: https://www.ncbi.nlm.nih.gov/pubmed/34324392 https://ascopubs.org/doi/abs/10.1200/JCO.21.00389