scholarly journals Recent Advances in Diagnosis and Therapy of Angioimmunoblastic T Cell Lymphoma

2021 ◽  
Vol 28 (6) ◽  
pp. 5480-5498
Author(s):  
Mostafa F. Mohammed Saleh ◽  
Ahmed Kotb ◽  
Ghada E. M. Abdallah ◽  
Ibrahim N. Muhsen ◽  
Riad El Fakih ◽  
...  

Angioimmunoblastic T cell lymphoma (AITL) is a common subtype of mature peripheral T cell lymphoma (PTCL). As per the 2016 World Health Organization classification, AITL is now considered as a subtype of nodal T cell lymphoma with follicular helper T cells. The diagnosis is challenging and requires a constellation of clinical, laboratory and histopathological findings. Significant progress in the molecular pathophysiology of AITL has been achieved in the past two decades. Characteristic genomic features have been recognized that could provide a potential platform for better diagnosis and future prognostic models. Frontline therapy for AITL was mainly depending on chemotherapy and the management of relapsed or refractory AITL is still unsatisfactory with a very poor prognosis. Upfront transplantation offers better survival. Novel agents have been introduced recently with promising outcomes. Several clinical trials of combinations using novel agents are underway. Herein, we briefly review recent advances in AITL diagnosis and the evolving treatment landscape.

2009 ◽  
Vol 133 (2) ◽  
pp. 303-308 ◽  
Author(s):  
Zahida Parveen ◽  
Karen Thompson

Abstract Subcutaneous panniculitis-like T-cell lymphoma is a primary T-cell lymphoma that preferentially involves the subcutaneous tissue. Although subcutaneous panniculitis-like T-cell lymphoma has been recognized as a distinctive entity in the category of peripheral T-cell lymphoma in the World Health Organization classification, its diagnostic criteria has been redefined by the recent World Health Organization–European Organization for Research and Treatment of Cancer classification for primary cutaneous lymphomas. Subcutaneous panniculitis-like T-cell lymphoma is now restricted to primary cutaneous T-cell lymphoma expressing αβ T-cell receptor phenotype. These lymphomas are usually CD3+, CD4−, CD8+, and CD56−, and usually have an indolent clinical course. The clinicopathologic features, differential diagnosis, immunophenotypic characteristics, and molecular features of subcutaneous panniculitis-like T-cell lymphoma are presented in light of the recent World Health Organization–European Organization for Research and Treatment of Cancer classification.


2018 ◽  
Vol 142 (11) ◽  
pp. 1347-1351 ◽  
Author(s):  
Steven C. Weindorf ◽  
Lauren B. Smith ◽  
Scott R. Owens

Herein we review the following selection of gastrointestinal lymphomas: monomorphic epitheliotropic intestinal T-cell lymphoma; indolent T-cell lymphoproliferative disorder of the gastrointestinal tract; intestinal T-cell lymphoma, not otherwise specified; duodenal-type follicular lymphoma; and Epstein-Barr virus–positive mucocutaneous ulcer. Definitions reflect the 2016 revision of the World Health Organization classification of lymphoid neoplasms. Clinical, morphologic, and immunophenotypic characteristics of each entity are emphasized.


2017 ◽  
Vol 141 (11) ◽  
pp. 1469-1475 ◽  
Author(s):  
Vivian M. Hathuc ◽  
Alexandra C. Hristov ◽  
Lauren B. Smith

Primary cutaneous acral CD8+ T-cell lymphoma is a new provisional entity in the 2016 revision of the World Health Organization classification of lymphoid neoplasms. This is a challenging diagnosis because of its rarity, as well as its morphologic and immunophenotypic overlap with other CD8+ cytotoxic lymphoid proliferations. Appropriate classification of this entity is crucial because of its indolent clinical behavior compared with other CD8+ T-cell lymphomas. Knowledge of the clinical setting, sites of involvement, and morphologic features can aid in correct diagnosis. Here, we review the clinical and pathologic features of primary cutaneous acral CD8+ T-cell lymphoma with an emphasis on the differential diagnosis among other C8+ T-cell lymphomas.


2018 ◽  
Vol 27 (1) ◽  
pp. 102-107 ◽  
Author(s):  
Anamarija M. Perry ◽  
Nathanael G. Bailey ◽  
Michelle Bonnett ◽  
Elaine S. Jaffe ◽  
Wing C. Chan

Indolent T-cell lymphoproliferative disorder (T-LPD) of the gastrointestinal (GI) tract is a new provisional entity in the 2016 revision of the World Health Organization classification. The disease has an indolent course and progression to aggressive T-cell lymphoma has rarely been reported. We describe a case of a 37-year-old male with indolent T-LPD of the GI tract who 3 years later developed aggressive T-cell lymphoma and died of progressive disease. An infiltrate of indolent T-LPD in the GI tract and aggressive lymphoma diagnosed from the liver biopsy had similar immunophenotype, but cellular infiltrate in the liver showed more atypia compared with the GI biopsies of indolent T-LPD. Moreover, T-cell gene rearrangement studies showed an identical clonal rearrangement in indolent T-LPD and aggressive lymphoma. Patients with indolent T-LPD of the GI tract need a long-term follow-up, as some of them may develop more aggressive lymphoma.


Blood ◽  
2017 ◽  
Vol 129 (9) ◽  
pp. 1095-1102 ◽  
Author(s):  
Matthew A. Lunning ◽  
Julie M. Vose

AbstractAngioimmunoblastic T-cell lymphoma (AITL) is an uncommon subtype of mature peripheral T-cell lymphoma (PTCL). The history of AITL is much longer and deeper than the literature would suggest given the many names that have preceded it. Advanced-stage disease is common with uncharacteristic laboratory and autoimmune findings that often slow or mask the diagnosis. Significant strides in the immunohistochemical and molecular signature of AITL have brought increased ability to diagnose this uncommon type of PTCL. The 2016 World Health Organization classification of lymphoid neoplasms recently acknowledged the complexity of this diagnosis with the addition of other AITL-like subsets. AITL now resides under the umbrella of nodal T-cell lymphomas with follicular T helper phenotype. Induction strategies continue to focus on increasing complete remission rates that allow more transplant-eligible patients to proceed toward consolidative high-dose therapy and autologous stem cell rescue with improving long-term survival. There are several clinical trials in which recently approved drugs with known activity in AITL are paired with induction regimens with the hope of demonstrating long-term progression-free survival over cyclophosphamide, doxorubicin, vincristine, and prednisone. The treatment of relapsed or refractory AITL remains an unmet need. The spectrum of AITL from diagnosis to treatment is reviewed subsequently in a fashion that may one day lead to personalized treatment approaches in a many-faced disease.


Author(s):  
Raul S. Gonzalez ◽  
Anwar Raza ◽  
Robert Propst ◽  
Oyedele Adeyi ◽  
Justin Bateman ◽  
...  

Context.— The World Health Organization Classification of Tumors: Digestive System Tumors, 5th edition, was published in 2019 and shows several impactful changes as compared with the 4th edition published in 2010. Changes include a revised nomenclature of serrated lesions and revamping the classification of neuroendocrine neoplasms. Appendiceal goblet cell adenocarcinoma is heavily revised, and intrahepatic cholangiocarcinoma is split into 2 subtypes. New subtypes of colorectal carcinoma and hepatocellular carcinoma are described. Precursor lesions are emphasized with their own entries, and both dysplastic and invasive lesions are generally recommended to be graded using a 2-tier system. Hematolymphoid tumors, mesenchymal tumors, and genetic tumor syndromes each have their own sections in the 5th edition. New hematolymphoid lesions include monomorphic epitheliotropic intestinal T-cell lymphoma; duodenal-type follicular lymphoma; intestinal T-cell lymphoma, not otherwise specified; and indolent T-cell lymphoproliferative disorder of the gastrointestinal tract. This paper will provide an in-depth look at the changes in the 5th edition as compared with the 4th edition. Objective.— To provide a comprehensive, in-depth update on the World Health Organization classification of digestive tumors, including changes to nomenclature, updated diagnostic criteria, and newly described entities. Data Sources.— The 5th edition of the World Health Organization Classification of Tumours: Digestive System Tumours, as well as the 4th edition. Conclusions.— The World Health Organization has made many key changes in its newest update on tumors of the digestive system. Pathologists should be aware of these changes and incorporate them into their practice as able or necessary.


2014 ◽  
Vol 3 (4) ◽  
pp. 191-200
Author(s):  
Bernardo Garicochea ◽  
Alessandro Igor Leal ◽  
Fernando Sérgio Fernando Sérgio Blumm Ferreira ◽  
Volney Vilela ◽  
Alesso Sartorelli ◽  
...  

2016 ◽  
Vol 19 (8) ◽  
pp. 897-906 ◽  
Author(s):  
Birgitt Wolfesberger ◽  
Ondrej Skor ◽  
Sabine E Hammer ◽  
Irene Flickinger ◽  
Miriam Kleiter ◽  
...  

Objectives The purpose of this study was to specify lymphoma subtypes according to the World Health Organization (WHO) classification in a group of cats and to investigate their potential prognostic value. Methods Records of cats from the University of Veterinary Medicine Vienna suffering from lymphoma were reviewed in this retrospective study. To diagnose various subtypes specified in the WHO classification, histopathological and immunohistochemical examinations, as well as clonality assays in some cases, were performed. Results Of the 30 cats included in this study and classified according to the WHO guidelines, peripheral T-cell lymphoma was the most prevalent lymphoma subtype (37% of cases; n = 11), followed by diffuse large B-cell (23%; n = 7), intestinal T-cell (10%; n = 3), T-cell-rich B-cell (10%; n = 3), large granular lymphocytic (7%; n = 2), anaplastic large T-cell (7%; n = 2), B-cell small lymphocytic (3%; n = 1) and T-cell angiotropic lymphoma (3%; n = 1). The median survival time (MST) was 5.4 months (range 6 days to 2.2 years), with two cats still alive after 1.7 and 2.0 years, respectively. Treating cats prior to chemotherapy with glucocorticoids did not worsen their prognosis. Adding to chemotherapy, radiotherapy or surgery did not improve the clinical outcome. We observed that patients with intestinal T-cell lymphoma lived significantly longer (MST 1.7 years) than those with a diffuse large B-cell (MST 4.5 months) or peripheral T-cell lymphoma (MST 6.1 months). Cats with T-cell-rich B-cell lymphoma survived significantly longer (MST 1.2 years) than those with a diffuse large B-cell lymphoma. Conclusions and relevance A detailed diagnosis of feline lymphoma can be obtained by allocating different subtypes according to the WHO classification. From the eight detected lymphoma subtypes, two, intestinal T-cell lymphoma and T-cell-rich B-cell lymphoma, showed promising survival times in cats.


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