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    Hypercalcemia as a rare presentation of angioimmunoblastic T cell lymphoma: a case report.
    J Med Case Rep 2018 Apr 20;12(1):101. Epub 2018 Apr 20.
    Department of Internal Medicine, Wayne State University School of Medicine, Rochester Hills, MI, USA.
    Background: Angioimmunoblastic T cell lymphoma is a rare malignancy, accounting for only 2% of all non-Hodgkin lymphomas, first described in the 1970s and subsequently accepted as a distinct entity in the current World Health Organization classification. Due to the paucity of this disease, there is still no identifiable etiology, no consistent risk factors, and the pathogenesis remains unclear.

    Case Presentation: An 83-year-old Caucasian man presented to an emergency department with palpitations and was found to have atrial fibrillation. Read More

    Novel insights into the pathogenesis of T-cell lymphomas.
    Blood 2018 Apr 17. Epub 2018 Apr 17.
    Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States
    T-cell lymphomas are a heterogeneous group of rare malignancies with overlapping clinical, immunologic and histologic features. Recent advances in our understanding of T-cell differentiation based on gene expression profiling, next-generation sequencing (NGS) and transgenic mouse modeling studies have better elucidated the pathogenetic mechanisms underlying the diverse biology of T-cell lymphomas. These studies show that while genetic alterations in epigenetic modifiers are implicated in all subtypes of T-cell lymphomas, specific subtypes demonstrate enrichment for particular recurrent alterations targeting specific genes. Read More

    Overlap at the molecular and immunohistochemical levels between angioimmunoblastic T-cell lymphoma and a subgroup of peripheral T-cell lymphomas without specific morphological features.
    Oncotarget 2018 Mar 1;9(22):16124-16133. Epub 2018 Mar 1.
    Pathology Department, Fundación Jiménez Díaz, UAM, Madrid, Spain.
    The overlap of morphology and immunophenotype between angioimmunoblastic T-cell lymphoma (AITL) and other nodal peripheral T-cell lymphomas (n-PTCLs) is a matter of current interest whose clinical relevance and pathogenic background have not been fully established. We studied a series of 98 n-PTCL samples (comprising 57 AITL and 41 PTCL-NOS) with five T antibodies (CD10, BCL-6, PD-1, CXCL13, ICOS), looked for mutations in five of the genes most frequently mutated in AITL (, and ) using the Next-Generation-Sequencing Ion Torrent platform, and measured the correlations of these characteristics with morphology and clinical features. The percentage of mutations in the and genes was similar (23. Read More

    Angioimmunoblastic T-cell lymphoma: a rare subtype of peripheral T-cell lymphoma.
    Clin Case Rep 2018 Apr 10;6(4):750-752. Epub 2018 Feb 10.
    Michigan State University/Sparrow hospitalB301, 788 Service RoadEast LansingMichigan48824.
    Angioimmunoblastic T-cell lymphoma (AITL) is a rare form of NHL and usually presents in the late stage due to the atypical laboratory findings. Immunohistochemistry of the lymph node in AITL is characterized by positive CD2, CD3, CD4, CD10, CXCL-13, PD1 often BCL-6 and CD20 positive. Meshworks of follicular dendritic cells are seen outside follicles with CD21 and CD23 stains. Read More

    Breast Implant-Associated Bilateral B-Cell Lymphoma.
    Aesthet Surg J 2018 Apr 9. Epub 2018 Apr 9.
    Department of Plastic and Reconstructive Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA.
    Lymphomas associated with implants are predominantly of T-cell type-with anaplastic large cell lymphoma (ALCL) being most reported. That said, to date 6 cases of B-cell lymphoma associated with breast implants have been reported. All cases exhibited unilateral breast involvement. Read More

    A Survey of Somatic Mutations in 41 Genes in a Cohort of T-Cell Lymphomas Identifies Frequent Mutations in Genes Involved in Epigenetic Modification.
    Appl Immunohistochem Mol Morphol 2018 Apr 7. Epub 2018 Apr 7.
    Department of Pathology, Stanford University, Stanford, CA.
    Here, we utilize a high throughput sequencing panel that covers several genes known to be recurrently mutated in certain T-cell lymphoma subtypes as well as genes frequently mutated in other hematolymphoid malignancies, including myeloid neoplasms. This panel was applied to formalin-fixed, paraffin-embedded tissue from 84 biopsies from 78 patients selected for this study. The biopsies included ones a with a diagnosis of T-cell lymphoma (n=79), including peripheral T-cell lymphoma not otherwise specified (PTCL-NOS; n=26) and angioimmunoblastic T-cell lymphoma (AITL; n=13), as well as 5 cases of atypical T-cell proliferations. Read More

    The Prognostic Role of Circulating Epstein-Barr Virus DNA Copy Number in Angioimmunoblastic T-Cell Lymphoma Treated with Dose-Adjusted EPOCH.
    Cancer Res Treat 2018 Apr 2. Epub 2018 Apr 2.
    Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China.
    Purpose: Determine the frequency and prognostic value of circulating Epstein-Barr virus (EBV) DNA copy number in angioimmunoblastic T-cell lymphoma (AITL) patients who were treated with dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin (DA-EPOCH) regimens.

    Materials And Methods: Sixty newly-diagnosed AITL patients were retrospectively enrolled in the present study. All patients were treated with DA-EPOCH regimen. Read More

    Mechanisms of Immune Evasion and Immune Modulation by Lymphoma Cells.
    Front Oncol 2018 7;8:54. Epub 2018 Mar 7.
    Institute of Pathology and Medical Genetics, University Hospital of Basel, Basel, Switzerland.
    Purpose: Targeting cancer cells by modulating the immune system has become an important new therapeutic option in many different malignancies. Inhibition of CTLA4/B7 and PD1/PDL1 signaling is now also being investigated and already successfully applied to various hematologic malignancies.

    Methods: A literature review of PubMed and results of our own studies were compiled in order to give a comprehensive overview on this topic. Read More

    Aggressive Angioimmunoblastic T Cell Lymphomas (AITL) with Soft Tissue Extranodal Mass Varied Histopathological Patterns with Peripheral Blood, Bone Marrow, and Splenic Involvement and Review of Literature.
    Indian J Surg Oncol 2018 Mar 18;9(1):11-14. Epub 2017 May 18.
    CHEC Kolkata, Kolkata, India.
    Angioimmunoblastic T cell lymphoma (AITL) is a peripheral T cell non-Hodgkin lymphoma with an aggressive fatal course and it has varied clinical presentation with an uncommon presentation when they present as soft tissue masses or when there is spill in the peripheral blood or there are composite lymphomas that are rare presentations. Common presentations include lymphadenopathy, fever and systemic symptoms, hemolytic anemias, skin rashes, and rheumatoid arthritis. The classical histopathology is absence of follicles in lymph nodes with presence of high endothelial venules and the tumor cells of small to medium-sized lymphocytes with pale cytoplasm mixed with reactive T cells. Read More

    [Expressions and clinical significance of GAS1, IL-1RAP and PRF1 in patients with ALK positive anaplastic large cell lymphoma].
    Zhonghua Xue Ye Xue Za Zhi 2018 Feb;39(2):116-121
    Department of Hematology and Research Laboratory of Hematology, West China Hospital of Sichuan University, Chengdu 610041, China.
    To investigate the expressions of growth arrest-specific protein (GAS1), IL-1 receptor accessory protein (IL-1RAP) and perforin (PRF1) in patients with anaplastic lymphoma kinase positive, anaplastic large cell lymphoma (ALK ALCL) and their relationships with clinical significances and the prognoses of ALK ALCL. Twenty-six formalin-fixed paraffin-embedded (FFPE) samples of ALK ALCL patients who were diagnosed from January 2011 to September 2016 were collected. Twelve FFPE samples of patients with ALKALCL, 13 FFPE samples of patients with peripheral T cell lymphoma (not otherwise specified) (PTCL-NOS) and 8 FFPE samples of patients with angioimmunoblastic T-cell lymphoma (AITL) were used as control groups. Read More

    Clinical and misdiagnosed analysis of primary pulmonary lymphoma: a retrospective study.
    BMC Cancer 2018 03 12;18(1):281. Epub 2018 Mar 12.
    The First Affiliated Hospital of Wenzhou Medical University & Key Laboratory of Heart and Lung, Zhejiang, 325035, China.
    Background: The primary pulmonary lymphoma (PPL), with a low incidence, was highly misdiagnosed in clinic. The present study analyzes the clinical features, laboratory and imaging data, pathologic characteristics, and summarizes misdiagnosis reasons of PPL cases, aims to provide a better understanding and increase the accuracy of early diagnosis and minimize the misdiagnosis of PPL.

    Methods: The clinical data of 19 cases were collected from the first affiliated hospital of Wenzhou medical university (PRC) from April 2010 to May 2016. Read More

    Droplet digital polymerase chain reaction assay and peptide nucleic acid-locked nucleic acid clamp method for RHOA mutation detection in angioimmunoblastic T-cell lymphoma.
    Cancer Sci 2018 Mar 1. Epub 2018 Mar 1.
    Department of Hematology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan.
    Angioimmunoblastic T-cell lymphoma (AITL) is a subtype of nodal peripheral T-cell lymphoma (PTCL). Somatic RHOA mutations, most frequently found at the hotspot site c.50G > T, p. Read More

    Therapeutic effect of chidamide on relapsed refractory angioimmunoblastic T-cell lymphoma: A case report and literature review.
    Medicine (Baltimore) 2018 Jan;97(2):e9611
    Department of Hematology, Wenzhou Central Hospital, Theorem Clinical College, Wenzhou Medical University, Wenzhou, P.R. China.
    Introduction: Angioimmunoblastic T-cell lymphoma (AITL) is a kind of rare peripheral T cell lymphoma, which usually has acute onset at old age.

    Materials And Methods: Here we report a case of relapsed refractory AITL, which has achieved obvious curative effect after treatment with chidamide.

    Results: Initially, the patient received 7 courses of treatment with recombinant human endostatin (endostar)+CHOP. Read More

    Emergence of anti-mitochondrial M2 antibody in patient with angioimmunoblastic T-cell lymphoma.
    Clin J Gastroenterol 2018 Feb 10. Epub 2018 Feb 10.
    Division of Gastroenterology, Department of Internal Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan.
    A 68-year-old woman was referred to our hospital due to fever and rash on the neck and extremities. Laboratory findings revealed hepatic dysfunction and positivity for anti-mitochondrial M2 antibody (AMA-M2). Hepatosplenomegaly and systemic lymphadenopathy were detected by enhanced computed tomography. Read More

    RHOA G17V Induces T Follicular Helper Cell Specification and Promotes Lymphomagenesis.
    Cancer Cell 2018 Feb 2;33(2):259-273.e7. Epub 2018 Feb 2.
    Institute for Cancer Genetics, Columbia University, New York, NY 10032, USA; Department of Pathology and Cell Biology, Columbia University Medical Center, 1130 St Nicholas Avenue, ICRC-401B, New York, NY 10032, USA. Electronic address:
    Angioimmunoblastic T cell lymphoma (AITL) is an aggressive tumor derived from malignant transformation of T follicular helper (Tfh) cells. AITL is characterized by loss-of-function mutations in Ten-Eleven Translocation 2 (TET2) epigenetic tumor suppressor and a highly recurrent mutation (p.Gly17Val) in the RHOA small GTPase. Read More

    [Research Progress on EB Virus and Its Related Lymphoma - Review].
    Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Feb;26(1):292-295
    Department of Hematology, Tongji Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China. E-mail:
    Epstein-Barr virus(EBV) closely relates with the occurrence, development and prognosis of multiple lymphomas, such as Burkitt' s lymphoma, diffuse large B-cell lymphoma, Hodgkin' s lymphoma, angioimmunoblastic T cell lymphoma. The mechanism of EBV in tumorigenesis can be understanded through the research of EBV biological characteristics, EBV lymphoma and EBV lymphoma. Based on traditional therapy, supplement of specific treatment for EBV, such as antiviral treatment, immunotherapy, can improve the curative rate of lymphoma. Read More

    Expression of activating natural killer-cell receptors is a hallmark of the innate-like T-cell neoplasm in peripheral T-cell lymphomas.
    Cancer Sci 2018 Apr 15;109(4):1254-1262. Epub 2018 Feb 15.
    Division of Hematology & Oncology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan.
    Peripheral T- or natural killer (NK)-cell lymphomas are rare and difficult-to-recognize diseases. It remains arduous to distinguish between NK cell- and cytotoxic T-lymphocyte-derived lymphomas through routine histological evaluation. To clarify the cells of origin, we focused on NK-cell receptors and examined the expression using immunohistochemistry in 22 cases with T- and NK-cell neoplasms comprising angioimmunoblastic T-cell lymphoma, anaplastic lymphoma kinase (ALK)-positive and -negative anaplastic large-cell lymphomas, extranodal NK/T-cell lymphoma, nasal type, monomorphic epitheliotropic intestinal T-cell lymphoma, aggressive NK-cell leukemia, and other peripheral T-cell lymphomas. Read More

    Comparison of Epstein-Barr virus-positive mucocutaneous ulcer associated with treated lymphoma or methotrexate in Japan.
    Histopathology 2018 Jan 4. Epub 2018 Jan 4.
    Department of Pathology and Molecular Diagnostics, Aichi Cancer Centre Hospital, Aichi, Japan.
    Aims: The aim of the present study was to compare treated lymphoma-associated Epstein-Barr virus (EBV)-positive mucocutaneous ulcer (EBVMCU) and methotrexate (MTX)-associated EBVMCU.

    Methods And Results: Of a series of 15 Japanese patients (11 women, four men; median age 74 years, range 35-84 years), seven received MTX for the treatment of autoimmune disease and eight developed EBVMCU after treatment of malignant lymphoma [diffuse large B-cell lymphoma (n = 4) without EBV association, adult T-cell leukaemia/lymphoma (n = 2), angioimmunoblastic T-cell lymphoma (n = 1), and follicular lymphoma (n = 1)]. Ulcers were observed in the oral cavity (n = 11), gastrointestinal tract (n = 2), and skin (n = 2). Read More

    Recent Progress in the Understanding of Angioimmunoblastic T-cell Lymphoma.
    J Clin Exp Hematop 2017 ;57(3):109-119
    Department of Hematology, Graduate School of Comprehensive Human Sciences, University of Tsukuba.
    Angioimmunoblastic T-cell lymphoma (AITL) has been classified as a subtype of mature T-cell neoplasms. The recent revision of the WHO classification proposed a new category of nodal T-cell lymphoma with follicular helper T (TFH)-cell phenotype, which was classified into three diseases: AITL, follicular T-cell lymphoma, and nodal peripheral T-cell lymphoma with TFH phenotype. These lymphomas are defined by the expression of TFH-related antigens, CD279/PD-1, CD10, BCL6, CXCL13, ICOS, SAP, and CXCR5. Read More

    Role of up-front autologous stem-cell transplantation in peripheral T-cell lymphoma for patients in response after induction: an analysis of patients from LYSA centers.
    Ann Oncol 2018 Mar;29(3):715-723
    Hematology Department, CHU Lyon Sud, Hospices Civils de Lyon, Pierre Benite, France.
    Background: Peripheral T-cell lymphoma (PTCL) remains a therapeutic challenge. Due to the rarity and the heterogeneity of PTCL, no consensus has been achieved regarding even the type of first-line treatment. The benefit of autologous stem-cell transplantation (ASCT) is, therefore, still intensely debated. Read More

    Quantitative Analysis of a Multiplexed Immunofluorescence Panel in T-Cell Lymphoma.
    SLAS Technol 2018 Mar 1:2472630317747197. Epub 2018 Mar 1.
    3 Cancer Science Institute of Singapore, National University of Singapore, Singapore.
    Immunohistochemistry (IHC) provides clinically useful information on protein expression in cancer cells. However, quantification of colocalizing signals using conventional IHC and visual scores is challenging. Here we describe the application of quantitative immunofluorescence in angioimmunoblastic T-cell lymphoma (AITL), a peripheral T-cell lymphoma characterized by cellular heterogeneity that impedes IHC interpretation and quantification. Read More

    Identification of autofluorescent cells in human angioimmunoblastic T-cell lymphoma.
    Histochem Cell Biol 2018 Feb 2;149(2):169-177. Epub 2017 Dec 2.
    Institute for Hematopathology, Fangdieckstreet 75a, 22547, Hamburg, Germany.
    Endogenous cell autofluorescence is a common nuisance that complicates the use of fluorescence microscopy. When using fluorescence-labeled antibodies for specific cell labeling in tissue sections of human angioimmunoblastic T-cell lymphoma (AITL), we encountered with a problematic autofluorescence of multiple cells. These cells emitted fluorescence signals in the green, red and deep-red spectral range. Read More

    Incidence and ten-year follow-up of primary cutaneous lymphomas: a single-centre cohort study.
    Eur J Dermatol 2018 Feb;28(1):44-49
    Department of Medicine, Section of Dermatology and Venereology, University of Verona, Verona, Italy.
    Background: Primary cutaneous lymphomas (PCLs) are a rare group of extranodal non-Hodgkin lymphomas, and epidemiological data in Mediterranean countries are scarce.

    Objective: To investigate the incidence and characteristics of PCL in a single tertiary referral centre in Italy.

    Materials & Methods: A total of 141 PCL patients, seen over a 10-year follow-up period, were investigated. Read More

    Indolent T-lymphoblastic proliferation concomitant with acinic cell carcinoma mimicking T-lymphoblastic lymphoma: case report and literature review.
    Histopathology 2018 Apr 19;72(5):862-866. Epub 2018 Jan 19.
    Department of Haematology, Juntendo University School of Medicine, Tokyo, Japan.
    Aims: Indolent T-lymphoblastic proliferation (iT-LBP) is a non-clonal benign condition showing extrathymic proliferation of T-lymphoblasts positive for CD3, CD4, CD8, and TdT. Isolated iT-LBP has been observed, but the majority of iT-LBPs have been seen in conjunction with other disorders, including Castleman disease, hepatocellular carcinoma, follicular dendritic cell tumours, angioimmunoblastic T-cell lymphoma, myasthenia gravis, and acinic cell carcinoma (ACC). The clinical course of iT-LBP is indolent, and no therapy is usually required. Read More

    Programmed death-1 ligands PD-L1 and PD-L2 show distinctive and restricted patterns of expression in lymphoma subtypes.
    Hum Pathol 2018 Jan 6;71:91-99. Epub 2017 Nov 6.
    Department of Pathology, Stanford University School of Medicine, Stanford, CA 94305, USA. Electronic address:
    The success of immunotherapy using immune checkpoint blockade in solid tumors and in relapsed/refractory classical Hodgkin lymphoma and chronic lymphocytic leukemia holds promise for targeted therapy in hematologic malignancies. Because efficacy of immunomodulatory therapy is correlated with numbers of cells that express programmed death (PD-1) ligands, we evaluated the expression of PD-L1 and PD-L2 proteins using immunohistochemistry in more than 702 diagnostic lymphoma biopsies. In classical Hodgkin lymphoma, PD-L1 and PD-L2 were expressed in 82% and 41% of cases, respectively, and PD-L1 but not PD-L2 expression correlated with Epstein-Barr virus in tumor cells. Read More

    Outcomes following front-line chemotherapy in peripheral T-cell lymphoma: 10-year experience at The Royal Marsden and The Christie Hospital.
    Leuk Lymphoma 2017 Nov 9:1-10. Epub 2017 Nov 9.
    b The University of Manchester and The Christie NHS Foundation Trust, Manchester Academic Health Science Centre , Manchester , UK.
    We evaluated the outcomes for patients with peripheral T-cell lymphoma (PTCL) undergoing front-line chemotherapy at our institutions between 2002 and 2012. One hundred and fifty-six patients were eligible, comprising PTCL not otherwise specified (NOS) (n = 50, 32.0%), angioimmunoblastic T-cell lymphoma (AITL) (n = 44, 28. Read More

    Lymphoproliferative disorders with concurrent HHV8 and EBV infection: beyond primary effusion lymphoma and germinotropic lymphoproliferative disorder.
    Histopathology 2018 Apr 3;72(5):855-861. Epub 2018 Jan 3.
    Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
    Aims: Lymphoproliferative disorders (LPD) characterised by human herpesvirus 8 (HHV8) and Epstein-Barr virus (EBV) infection are rare and two entities are included in the World Health Organisation classification: primary effusion lymphoma (PEL) and germinotropic LPD. These two entities have very different clinicopathological presentations and prognosis. Here we describe two cases of HHV8-positive, EBV-positive lymphoma with clinicopathological features that are not consistent with either PEL or germinotropic LPD. Read More

    Elevated serum interleukin-10 level and M2 macrophage infiltration are associated with poor survival in angioimmunoblastic T-cell lymphoma.
    Oncotarget 2017 Sep 17;8(44):76231-76240. Epub 2017 Jul 17.
    Division of Hematology and Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
    Interleukin-10 (IL-10) induces an immunosuppressive microenvironment including M2 macrophages, inhibiting anti-tumor immunity. The aim of this study was to evaluate whether serum IL-10 level at diagnosis and tissue infiltration of M2 macrophages could predict survival outcome of patients with angioimmunoblastic T-cell lymphoma (AITL). We measured serum levels of IL-5, IL-10, IL-12, and interferon-gamma (IFN-γ) at diagnosis in AITL and other common subtypes of nodal T-cell lymphoma including peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), ALK-negative anaplastic large cell lymphoma (ALCL) or ALK-positive ALCL between September 2008 and December 2014. Read More

    Dose-adjusted EPOCH chemotherapy for untreated peripheral T-cell lymphomas: a multicenter phase II trial of West-JHOG PTCL0707.
    Haematologica 2017 Dec 29;102(12):2097-2103. Epub 2017 Sep 29.
    Department of Hematology and Oncology, Okayama University Hospital, Japan.
    The standard CHOP therapy for peripheral T-cell lymphoma has resulted in unsatisfactory outcomes and it is still not clear what is the optimal front-line therapy. We conducted a multicenter phase II study of dose-adjusted etoposide, doxorubicin, and cyclophosphamide with vincristine and prednisone (EPOCH) for untreated peripheral T-cell lymphoma patients. In this prospective study, 41 patients were treated with dose-adjusted-EPOCH as initial therapy: peripheral T-cell lymphoma-not otherwise specified, n=21; angioimmunoblastic T-cell lymphoma, n=17; anaplastic lymphoma kinase-positive anaplastic large cell lymphoma, n=2; and anaplastic lymphoma kinase-negative anaplastic large cell lymphoma, n=1. Read More

    Clinical characteristics of angioimmunoblastic T-cell lymphoma in China and C-reactive protein as an independent prognostic factor.
    Medicine (Baltimore) 2017 Sep;96(39):e8091
    aDepartment of Hematology, the First Affiliated Hospital of Zhejiang University bInstitute of Hematology, Zhejiang University, Hangzhou, Zhejiang Province, People's Republic of China.
    Angioimmunoblastic T-cell lymphoma (AITL) is a major subtype of peripheral T-cell lymphoma (PTCL). Due to its low incidence, the characteristics of AITL are still not well understood. The prognostic evaluation of this disease has not been established. Read More

    Expression of TFH Markers and Detection of RHOA p.G17V and IDH2 p.R172K/S Mutations in Cutaneous Localizations of Angioimmunoblastic T-Cell Lymphomas.
    Am J Surg Pathol 2017 Dec;41(12):1581-1592
    *Pathology Department, Henri Mondor Hospital, Assistance Publique-Hôpitaux de Paris †INSERM U955 équipe 9, Institut Mondor de Recherche Biomédicale ‡Paris Est Creteil University (UPEC) ¶Biological Immunology Department, Henri Mondor Hospital, Assistance Publique-Hôpitaux de Paris #Dermatology department, Henri Mondor Hospital, Assistance Publique-Hôpitaux de Paris **Hematology Department, Lymphoid hemopathy Unit, Henri Mondor Hospital, Assistance Publique-Hôpitaux de Paris, Créteil ††Dermatology Department, Saint-André Hospital, CHU de Bordeaux ‡‡INSERM U1053, Bordeaux Research in Translational Oncology, Team 3 Oncogenesis of Cutaneous Lymphomas, Bordeaux University, Bordeaux §Pathoogy department, Haut-Lévèque Hospital, CHU de Bordeaux §§Tumor Biology Department, Haut-Lévèque Hospital, CHU de Bordeaux, Pessac, France ∥Pathology Department Prof Lima Basto, Lisboa, Portugal.
    Skin biopsies of 41 angioimmunoblastic T-cell lymphoma patients were retrospectively analyzed for the expression of follicular helper T-cell (TFH) markers, Epstein-Barr virus (EBV), and the presence of RHOA (p.G17V) and IDH2 (p.R172K/S) mutations using allele-specific polymerase chain reaction. Read More

    CMIP is oncogenic in human gastric cancer cells.
    Mol Med Rep 2017 Nov 20;16(5):7277-7286. Epub 2017 Sep 20.
    Department of General Surgery, The First Affiliated Hospital of Anhui Medical University, Hefei, Anhui 230022, P.R. China.
    Gastric cancer is one of the most common cancers and the second leading cause of cancer‑associated mortality worldwide. Recurrence, metastasis and resistance to drug treatment are the main barrier to survival of patients with advanced stage gastric cancer. Further study of the molecular mechanisms involved will improve the therapeutic options for gastric cancer. Read More

    Expression of Master Regulators of T-cell, Helper T-cell and Follicular Helper T-cell Differentiation in Angioimmunoblastic T-cell Lymphoma.
    Intern Med 2017 Nov 25;56(21):2851-2856. Epub 2017 Sep 25.
    Departments of Hematology and Laboratory Medicine, Aiseikai Yamashina Hospital, Japan.
    Objective It has been postulated that the normal counterpart of angioimmunoblastic T-cell lymphoma (AITL) is the follicular helper T-cell (TFH). Recent immunological studies have identified several transcription factors responsible for T-cell differentiation. The master regulators associated with T-cell, helper T-cell (Th), and TFH differentiation are reportedly BCL11B, Th-POK, and BCL6, respectively. Read More

    Study of association of Epstein-Barr virus in lymphomas by Epstein-Barr virus-encoded RNA hybridization: An Indian perspective from a tertiary care cancer institute.
    Indian J Pathol Microbiol 2017 Jul-Sep;60(3):341-349
    Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India.
    Background: The Epstein-Barr virus (EBV), also called human herpesvirus 4, is a virus of the herpes family. The EBV-associated lymphomas include Burkitt lymphoma, classic Hodgkin lymphoma (HL), lymphomas arising in immunocompromised individuals, peripheral T-cell lymphomas, angioimmunoblastic T-cell lymphoma, extranodal nasal-type natural killer/T-cell lymphoma, and other rare histotypes.

    Objective: The present study evaluated the role of EBV as an etiologic agent in various lymphomas and determined an Indian perspective in a tertiary care cancer center compared to that of Western literature. Read More

    Review of the biologic and clinical significance of genetic mutations in angioimmunoblastic T-cell lymphoma.
    Cancer Sci 2018 Mar 27;109(3):490-496. Epub 2017 Nov 27.
    Department of Hematology, Graduate School of Comprehensive Human Sciences, University of Tsukuba Hospital, Tsukuba, Ibaraki, Japan.
    Angioimmunoblastic T-cell lymphoma (AITL) is an age-related malignant lymphoma, characterized by immune system-dysregulated symptoms. Recent sequencing studies have clarified the recurrent mutations in ras homology family member A (RHOA) and in genes encoding epigenetic regulators, tet methyl cytosine dioxygenase 2 (TET2), DNA methyl transferase 3 alpha (DNMT3A) and isocitrate dehydrogenase 2, mitochondrial (IDH2), as well as those related to the T-cell receptor signaling pathway in AITL. In this review, we focus on how this genetic information has changed the understanding of the developmental process of AITL and will in future lead to individualized therapies for AITL patients. Read More

    Angioimmunoblastic T-cell lymphoma and hypereosinophilic syndrome with FIP1L1/PDGFRA fusion gene effectively treated with imatinib: A case report.
    Medicine (Baltimore) 2017 Sep;96(36):e8001
    aDivision of Gastroenterology and Hematology/Oncology, Department of Medicine bOncology Center, Asahikawa Medical University Hospital, Asahikawa, Japan.
    Rationale: Hypereosinophilic syndrome (HES) is a rare disorder characterized by hypereosinophilia and organ damage. Some cases of HES are caused by the FIP1L1/PDGFRA fusion gene and respond to imatinib. FIP1L1/PDGFRA-positive HES occasionally evolves into chronic eosinophilic leukemia or into another form of myeloproliferative neoplasm; however, the development of a malignant lymphoma is very rare. Read More

    Activation of RHOA-VAV1 signaling in angioimmunoblastic T-cell lymphoma.
    Leukemia 2018 Mar 23;32(3):694-702. Epub 2017 Aug 23.
    Department of Hematology, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan.
    Somatic G17V RHOA mutations were found in 50-70% of angioimmunoblastic T-cell lymphoma (AITL). The mutant RHOA lacks GTP binding capacity, suggesting defects in the classical RHOA signaling. Here, we discovered the novel function of the G17V RHOA: VAV1 was identified as a G17V RHOA-specific binding partner via high-throughput screening. Read More

    Clinical Practice Recommendations on Indication and Timing of Hematopoietic Cell Transplantation in Mature T Cell and NK/T Cell Lymphomas: An International Collaborative Effort on Behalf of the Guidelines Committee of the American Society for Blood and Marrow Transplantation.
    Biol Blood Marrow Transplant 2017 Nov 7;23(11):1826-1838. Epub 2017 Aug 7.
    Vanderbilt University Medical Center, Nashville, Tennessee.
    Recognizing the significant biological and clinical heterogeneity of mature T cell and natural killer (NK)/T cell lymphomas, the American Society for Blood and Marrow Transplantation invited experts to develop clinical practice recommendations related to the role of autologous hematopoietic cell transplantation (auto-HCT) and allogeneic HCT (allo-HCT) for specific histological subtypes. We used the GRADE methodology to aid in moving from evidence to decision making and ultimately to generating final recommendations. Auto-HCT in front-line consolidation is recommended in peripheral T cell lymphoma not otherwise specified (PTCL-NOS), angioimmunoblastic T cell lymphoma (AITL), anaplastic large cell lymphoma-anaplastic lymphoma kinase (ALCL-ALK)-negative, NK/T cell (disseminated), enteropathy-associated T cell lymphoma (EATL), and hepatosplenic lymphomas. Read More

    Lymphocytic hidradenitis: A distinctive histopathological finding of annular erythema of Sjögren syndrome.
    Australas J Dermatol 2017 Aug 3. Epub 2017 Aug 3.
    College of Medicine, Chang Gung University, Taoyuan, Taiwan.
    Background/objectives: Lymphocytic hidradenitis is a non-specific histopathological feature observed in many dermatoses such as lupus erythematosus, morphea or scleroderma. When it occurs it is usually accompanied by the other distinctive histological features of those conditions. Isolated lymphocytic hidradenitis is uncommon and its clinical features and associated underlying medical conditions are still undetermined. Read More

    Functional analysis of acquired CD28 mutations identified in cutaneous T cell lymphoma.
    Cell Immunol 2017 Sep 10;319:28-34. Epub 2017 Jul 10.
    Washington University School of Medicine, St Louis, MO 63110, USA. Electronic address:
    CD28 is the major costimulatory receptor on T cells regulating proliferation, survival and effector function. Acquired mutations in the extracellular domain of CD28 have been identified in patients with cutaneous T cell lymphoma, angioimmunoblastic T cell lymphoma and other T cell neoplasms, suggesting it may contribute to disease pathogenesis. We used a heterologous system in which mutant human CD28 was expressed on primary murine T cells deficient in CD28 to ascertain how specific mutations identified in a genetic screen of patients with cutaneous T cell lymphoma affected normal T cell function. Read More

    Mutations in 5-methylcytosine oxidase TET2 and RhoA cooperatively disrupt T cell homeostasis.
    J Clin Invest 2017 Aug 10;127(8):2998-3012. Epub 2017 Jul 10.
    Center for Epigenetics and Disease Prevention, Institute of Biosciences and Technology and.
    Angioimmunoblastic T cell lymphoma (AITL) represents a distinct, aggressive form of peripheral T cell lymphoma with a dismal prognosis. Recent exome sequencing in patients with AITL has revealed the frequent coexistence of somatic mutations in the Rho GTPase RhoA (RhoAG17V) and loss-of-function mutations in the 5-methylcytosine oxidase TET2. Here, we have demonstrated that TET2 loss and RhoAG17V expression in mature murine T cells cooperatively cause abnormal CD4+ T cell proliferation and differentiation by perturbing FoxO1 gene expression, phosphorylation, and subcellular localization, an abnormality that is also detected in human primary AITL tumor samples. Read More

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