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precursor T-lymphoblastic leukemia/lymphoma

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: 1) serum LDH has prognostic value 2) lumbar puncture with CSF analysis 3) chromosome region 1p32 deletion STIL,TAL1 fusion portends good prognosis in children Management: 1) chemotherapy 2) central nervous system prophylaxis 3) donor-derived anti-CD7 CAR T-cell therapy - complete response in 90% of relapsed or refractory cases in small study [3]

Interactions

disease interactions

Specific

acute lymphoblastic leukemia with lymphomatous features; lymphomatous ALL

General

acute lymphoblastic leukemia (ALL) lymphoblastic lymphoma precursor T-cell lymphoid neoplasm

Database Correlations

OMIM correlations

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, American College of Physicians, Philadelphia 1998
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 519
  3. 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: 34324392 https://ascopubs.org/doi/abs/10.1200/JCO.21.00389