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Mediastinal tumours
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Lymphoblastic lymphoma is highly aggressive, arising from thymic lymphocytes (118). Common symptoms include cough, wheezing, shortness of breath, superior vena cava syndrome, cardiac tamponade, or tracheal obstruction and can involve the mediastinum, bone marrow, CNS, skin, or gonads (118). It is often confused with T-cell acute lymphoblastic leukaemia (ALL) because bone marrow involvement with blasts is relatively common (119,120).
Case 55
Published in Atul B. Mehta, Keith Gomez, Clinical Haematology, 2017
The blood film shows many leukaemic blast cells which are large and have relatively little cytoplasm, prominent nucleoli and convoluted nuclei. They show positivity for acid phosphatase at their poles (‘polar positivity’) and are likely to be T cells. The likeliest diagnosis is T-cell acute lymphoblastic leukaemia, which is often seen in this age group. Immunohistochemistry confirmed that the cells are positive for the enzyme terminal deoxynucleotidyl transferase (TdT) (Figure 55a) and have T-cell markers, for example, cluster of differentiation 3 (CD3) (Figure 55b). The cells are negative for B-cell markers, for example, CD 19 (Figure 55c). Flow cytometry should also be used to differentiate early T-cell precursor acute lymphoblastic leukaemia (ALL), which has a poorer prognosis and is positive only for cytoplasmic CD3 and CD7, from the much commoner thymic or cortical T-cell derived form of the disease. Flow cytometry is an effective way of monitoring treatment response and for detecting residual disease. These monoclonal T cells also have a discrete rearrangement of T-cell receptor genes, and this is a specific marker of the disease. It can be used to detect minimal residual disease after chemotherapy. Cytogenetic analysis should be undertaken so that a full analysis can be undertaken of the likely prognosis. Hyperdiploidy and the presence of the t(12;21) translocation indicate a good prognosis and are much rarer in adult ALL than in childhood. The presence of the Philadelphia chromosome (t(9;22)) is detected in up to 25% of adults; and its presence, or the presence of the (4;11) translocation, is associated with a poor prognosis; however, failure to clear leukaemic cells from marrow and blood for 4 weeks, and chromosome changes such as hypodiploidy would indicate a bad risk for disease.
Metformin induces myeloma cells necrosis and apoptosis and it is considered for therapeutic use
Published in Journal of Chemotherapy, 2023
Zhentian Wu, Lianghua Wu, Liangliang Zou, Muqing Wang, Xin Liu
It is reported that metformin’s antitumor effect is largely adenosine 5′-monophosphate (AMP)-activated protein kinase (AMPK)-dependent in various cancer types. In esophageal squamous cell carcinoma, metformin treatment suppresses the carcinogenesis through inhibiting AMPK/mammalian target of the rapamycin (mTOR) signaling pathway and thus induces cell apoptosis [16]. Furthermore, extensive research has shown the effect in other solid tumors, e.g. breast cancer, cervical cancer, prostate cancer, etc. [17–19]. Study on hematological malignance T-cell acute lymphoblastic leukemia shows the identical effect [20]. Notably, it takes little effect against proliferating of CD4+ T-lymphocytes from healthy donors [20]. Furthermore, in the assay from Philadelphia chromosome-positive leukemia cell line and patient primary leukemia cell, including imatinib resistant cells K562R, metformin is shown to have the anticancer potential through extracellular signal regulated kinases (ERK) pathway and induce apoptosis and autophage [21].
Ring chromosome 7 in a child with T-cell acute lymphoblastic leukemia with myeloid markers
Published in Baylor University Medical Center Proceedings, 2021
Carlos A. Tirado, Andrew Reyes, Wilson Yeh, Justin Yee, Joy King, Javier Kane, William Koss
T-cell acute lymphoblastic leukemia (T-ALL) is an aggressive hematologic malignancy resulting from the transformation and excessive proliferation of lymphoid T-cell progenitors. T-ALL accounts for 15% of all childhood cases of acute lymphoblastic leukemia (ALL) and 25% of all adult ALL cases, though the majority of T-ALL cases occur before age 40.1 Historically, T-ALL has been associated with poor clinical outcomes, but advances in intensified chemotherapy have raised pediatric and adult cure rates to over 80% and 50%, respectively.2 The presence of ring chromosomes (RCs) in T-ALL is rare, and as such their implications are not well understood within the context of this disease.3 We report a case of a girl diagnosed with T-ALL, in whom cytogenetics revealed the presence of ring 7 and deletions of the subtelomeric regions on chromosome 7.
The potential of precision medicine for childhood acute lymphoblastic leukemia: opportunities and challenges
Published in Expert Review of Precision Medicine and Drug Development, 2018
Nicole Mallory, Joanna Pierro, Elizabeth Raetz, William L. Carroll
The prognostic significance of clinical characteristics such as age and WBC count at diagnosis are less defined for T cell acute lymphoblastic leukemia (T-ALL) as opposed to B-ALL. Additionally, although there are several genomic alterations associated with T-ALL, the prognostic significance of these changes has been unclear and genetic features are not currently used for risk stratification in T-ALL. However, like B-ALL, the response to treatment (minimal residual disease (MRD) see below) is an important prognostic indicator in T-ALL and is the basis for risk stratification on current treatment protocols [7]. More recent data have highlighted the prognostic impact of somatic mutations in childhood T-ALL and may be incorporated into future risk stratification. For example, Petit et al characterized NOTCH1, FBXW7, K-RAS/N-RAS and PTEN alterations in 220 children treated on the FRALLE200T clinical trial [8]. Patients with NOTCH1/FBXW7 mutations without associated RAS and PTEN alterations fared well compared to those patients whose blasts contained RAS/PTEN alterations (regardless of NOTCH1/FBXW7 mutational status). The authors developed a risk stratifier combining WBC, mutation status and MRD status to classify patients into low risk (DFS 98.3%, CI 95.0–100), intermediate risk (DFS 70.9%, CI 61.0–82.5) and high risk (DFS 37.7%, CI 25.3–50.1). However, there was no prognostic significance of NOTCH1 mutations found in other trials [9] and application of the four gene signature developed by Petit et al did not show the same level of significance in the UKALL2003 trial [8,10].