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      A Challenging Case of Gamma Delta T-Cell Lymphoma with Precursor T-Cells and Marked Eosinophilia: A Case Report

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          Abstract

          Gamma-delta (γδ) T-cell lymphomas are very rare and aggressive neoplasms. We describe here a challenging case of γδ T-cell neoplasm composed of γδ mature T-cells and γδ precursor T-cells with marked eosinophilia that is inapplicable to the current 2016 World Health Organization (WHO) classification. A 3-year-old female child who was presented with fever and marked leukocytosis. Peripheral blood smear showed marked lymphocytosis, marked eosinophilia, neutrophilia, monocytosis, and 5% circulating blasts. CT scan showed anterior mediastinal mass, lymphadenopathy, and hepatosplenomegaly. The patient underwent a bone marrow examination and a biopsy taken from the mediastinal mass. Peripheral blood and bone marrow findings were consistent with a γδ T-cell neoplasm with increased blasts and eosinophilia. The patient was sequentially treated with imatinib (tyrosine kinase inhibitor), acute lymphoblastic leukemia protocol (BFM 2009) then shifted to lymphoma protocol (LMP 96). In conclusion, we report a unique rare case of γδ T-cell neoplasm with a combination of mature and immature γδ T-cells and eosinophilia that is inapplicable to the current 2016 WHO classifications. This case raises a challenging concept of a mature T-cell lymphoma arising in an immature T-cell neoplasm. It also highlights the need to target all neoplastic components to eradicate the disease.

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          Most cited references22

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          The 2016 revision of the World Health Organization classification of lymphoid neoplasms.

          A revision of the nearly 8-year-old World Health Organization classification of the lymphoid neoplasms and the accompanying monograph is being published. It reflects a consensus among hematopathologists, geneticists, and clinicians regarding both updates to current entities as well as the addition of a limited number of new provisional entities. The revision clarifies the diagnosis and management of lesions at the very early stages of lymphomagenesis, refines the diagnostic criteria for some entities, details the expanding genetic/molecular landscape of numerous lymphoid neoplasms and their clinical correlates, and refers to investigations leading to more targeted therapeutic strategies. The major changes are reviewed with an emphasis on the most important advances in our understanding that impact our diagnostic approach, clinical expectations, and therapeutic strategies for the lymphoid neoplasms.
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            From stem cell to T cell: one route or many?

            T cells developing in the adult thymus ultimately derive from haematopoietic stem cells in the bone marrow. Here, we summarize research into the identity of the haematopoietic progenitors that leave the bone marrow, migrate through the blood and settle in the thymus to generate T cells. Accumulating data indicate that various different bone-marrow progenitors are T-cell-lineage competent and might contribute to intrathymic T-cell development. Such developmental flexibility implies a mechanism of T-cell-lineage commitment that can operate on a range of T-cell-lineage-competent progenitors, and further indicates that only those T-cell-lineage-competent progenitors able to migrate to, and settle in, the thymus should be considered physiological T-cell progenitors.
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              How I diagnose and treat splenic lymphomas.

              The incidental finding of an isolated splenomegaly during clinical assessment of patients evaluated for unrelated causes has become increasingly frequent because of the widespread use of imaging. Therefore, the challenging approach to the differential diagnosis of spleen disorders has emerged as a rather common issue of clinical practice. A true diagnostic dilemma hides in distinguishing pathologic conditions primarily involving the spleen from those in which splenomegaly presents as an epiphenomenon of hepatic or systemic diseases. Among the causes of isolated splenomegaly, lymphoid malignancies account for a relevant, yet probably underestimated, number of cases. Splenic lymphomas constitute a wide and heterogeneous array of diseases, whose clinical behavior spans from indolent to highly aggressive. Such a clinical heterogeneity is paralleled by the high degree of biologic variation in the lymphoid populations from which they originate. Nevertheless, the presenting clinical, laboratory, and pathologic features of these diseases often display significant overlaps. In this manuscript, we present our approach to the diagnosis and treatment of these rare lymphomas, whose complexity has been so far determined by the lack of prospectively validated prognostic systems, treatment strategies, and response criteria. © 2011 by The American Society of Hematology
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                Author and article information

                Journal
                CRO
                CRO
                10.1159/issn.1662-6575
                Case Reports in Ophthalmology
                S. Karger AG
                1662-6575
                2020
                September - December 2020
                18 December 2020
                : 13
                : 3
                : 1520-1529
                Affiliations
                [_a] aDepartment of Laboratory Medicine and Pathology, Hematology Section, NCCCR, Hamad Medical Corporation, Doha, Qatar
                [_b] bDepartment of Clinical Pathology, Hematology Division, Faculty of Medicine, Al-Azhar University, Cairo, Egypt
                [_c] cDepartment of Pediatric Hematology/Oncology, Hamad Medical Corporation, Doha, Qatar
                [_d] dDepartment of Laboratory Medicine and Pathology, Anatomic Pathology Section, Hamad Medical Corporation, Doha, Qatar
                [_e] eWeill Cornell Medicine, Doha, Qatar
                Author notes
                *Samah Kohla, Department of Laboratory Medicine and Pathology, Hematology Section, NCCCR/ORI, Hamad Medical Corporation, Al-Rayyan Street, Doha 3050 (Qatar), skohla@hamad.qa
                Author information
                https://orcid.org/0000-0002-1050-9922
                Article
                512188 Case Rep Oncol 2020;13:1520–1529
                10.1159/000512188
                a259bd2d-b1ab-4be3-8593-d50b6a20a14e
                © 2020 The Author(s). Published by S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 19 September 2020
                : 21 September 2020
                Page count
                Figures: 6, Pages: 10
                Categories
                Case Report

                Vision sciences,Ophthalmology & Optometry,Pathology
                γδ T-cell lymphoma,Lymphoid neoplasms associated with eosinophilia,Tyrosine kinase inhibitors,Eosinophilia,γδ T-cell neoplasm,T-lymphoblastic leukemia/lymphoma,T-cell lymphoma,Clonal eosinophilia

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