China
Africa
Explore chapters and articles related to this topic
Pediatric Oncology
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Stephen Lowis, Rachel Cox, John Moppett, Helen Rees
B-cell type lymphomas characteristically express CD20, CD19, CD10, and CD79a and may express surface immunoglobulin (sIg) and bcl-6. Translocations t(8;14), t(2;8), and t(8;22) and MYC rearrangements are typical. B-lymphoblastic lymphoma often shows IGH rearrangements, whilst T-lymphoblastic lymphoma show T-cell receptor rearrangements, and abnormalities of NOTCH/FBXW7 and PTEN pathways. ALCL typically express CD30 and carry a translocation causing fusion of the anaplastic lymphoma kinase (ALK) gene to neucleophosmin (NPM/ALK).
Other Myeloproliferative Neoplasms
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Onset of the EMS is acute without a prior history of cytotoxic therapy. The majority of patients have leukocytosis (median 46 × 109/L; range 3.8–400) with a leukemoid reaction, circulating blasts (55% of cases), eosinophilia (79%), and/or monocytosis (37%) [13]. The BM is usually hyper-cellular with myeloid hyperplasia and eosinophilia (71% of cases) and its morphologic features overlapping between MPN (with eosinophilia and/or mastocytosis), myelodys-plastic syndrome (MDS), and acute leukemia. In a series reported by Jackson et al. [13], 66% of cases had blast counts in the normal range, 18% had between 6% and 19%, and 16% had ≥20% blasts (usually myeloid or mixed phenotype lineage). In the lymph node, the majority of cases show T-lymphoblastic lymphoma (79%) and the remaining cases show myeloid sarcoma (21%) [15–17].
Investigational treatment options in phase I and phase II trials for relapsed or refractory acute lymphoblastic leukemia in pediatric patients
Published in Expert Opinion on Investigational Drugs, 2021
Julie M. Asare, Cara A. Rabik, Bradley Muller, Patrick A. Brown, Stacy Cooper
Currently, Texas Children’s Hospital has an open phase 1 study for anti-CD5 CAR T-cells for patients with relapsed or recurrent T-cell malignancies, including T-ALL and T-lymphoblastic lymphoma (T-Lly) [47]. Of note, preclinical studies showed only limited fratricide from co-expression of CD5 on CAR T-cells, without significant ex vivo limitations of expansion. As such, manipulation of the CAR T-cells to prevent fratricide was not undertaken in this protocol [47,48]. The Baylor College of Medicine will soon be opening a study of autologous T-cells directed against CD7, using CRISPR/Cas9 to remove CD7 from the CAR T-cells prior to infusion and prevent fratricide as described above [42,46]. As CD7 is present on a number of malignancies, T-ALL and T-NHL will be included in this study (estimated opening date of March 2021) [49].
Harnessing immunotherapy for pediatric T-cell malignancies
Published in Expert Review of Clinical Immunology, 2020
Caroline Diorio, David T. Teachey
T-cell malignancies are cancers of T-cell precursors. They can be divided broadly in to T-cell leukemias and peripheral T-cell lymphomas. In children, the most common T-cell malignancy is T-cell acute lymphoblastic leukemia (T-ALL), which represents approximately 15% of pediatric ALL cases[18]. Approximately one third of pediatric non-Hodgkin lymphomas are T-lymphoblastic lymphoma (T-LBL), which shares an immunophenotype with T-ALL[19]. T-ALL is distinguished from T-LBL based on the degree of bone marrow involvement: patients with T-LBL have <25% bone marrow involvement[19]. Pediatric T-ALL is a distinct entity from adult T-cell leukemia (ATL), which is a primarily human T cell leukemia/lymphotropic virus type 1 (HTLV-1) driven cancer[20]. HTLV-1 related leukemias are extremely rare in the pediatric population. By far the most common peripheral T-cell lymphoma in children is anaplastic large cell lymphoma (ALCL). In adults, cutaneous T-cell lymphomas make up a significant portion of T-cell malignancies; these conditions are very rarely seen in the pediatric population.
Pharmacotherapeutic management of T-cell acute lymphoblastic leukemia in adults: an update of the literature
Published in Expert Opinion on Pharmacotherapy, 2022
Alexander Grunenberg, Elisa Sala, Silke Kapp-Schwoerer, Andreas Viardot
T-Lymphoblastic lymphoma (LBL) is classified as T-ALL “solid” counterpart, and it shows frequent presence as a large mediastinal mass. In many treatment protocols, the cut off bone marrow infiltration is between 20% and 25% blasts [3]. T-LBLs have a higher incidence of thymic and mature immunophenotypic characteristics than their leukemic counterparts [14]. It is still a matter of debate if T-ALL and T-LBL are different presentations of the same disease, or if they show different evolutions of a common malignant precursor clone [15]. The treatment of T-LBL resembles standard T-ALL protocols. The prognosis is dependent on clinical risk factors like stage and lactate dehydrogenase serum levels.