Angioimmunoblastic T-Cell Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma: A Novel Form of Composite Lymphoma Potentially Mimicking Richter Syndrome. (June 2021)

• Record Type: Journal Article
• Authors: Trimech, Mounir; Letourneau, Audrey; Missiaglia, Edoardo; De Prijck, Bernard; Nagy-Hulliger, Monika; Somja, Joan; Vivario, Manuela; Gaulard, Philippe; Lambert, Frédéric; Bisig, Bettina; de Leval, Laurence
• Is Part Of: American journal of surgical pathology. Volume 45:Number 6(2021)
• Publication Date: 2021-06
• Subjects: composite lymphoma -- chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) -- angioimmunoblastic T-cell lymphoma (AITL) -- Richter syndrome -- T follicular helper (TFH) cell -- next-generation sequencing; Pathology, Surgical -- Periodicals; 617.0705
• DOI: 10.1097/PAS.0000000000001646
• Languages: English
• ISSNs: 0147-5185

Abstract

Abstract : Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is an indolent small B-cell neoplasm that may transform into a clinically aggressive disease, namely Richter syndrome, usually as diffuse large B-cell lymphoma. Besides, CLL/SLL encompasses an increased risk of developing other secondary cancers, including a variety of T-cell lymphomas, often of the anaplastic large-cell type or with a cytotoxic phenotype. Here, we report a small series of patients with composite lymphomas consisting of CLL/SLL and angioimmunoblastic T-cell lymphoma (AITL), a hitherto unrecognized association. The 3 patients (1 male/2 females, 68 to 83 y) presented with high-grade-type symptoms. One patient was clinically suspicious for Richter syndrome, in the others CLL/SLL and AITL were concomitant de novo diagnoses. CLL/SLL and AITL were admixed in the same lymph nodes (3/3 cases) and in the bone marrow (1/2 cases). In all cases, the AITL comprised prominent clear cells with a strong T follicular helper immunophenotype and similar mutations consisting of TET2 or DNMT3A alterations, IDH2 R172K/M, and RHOA G17V. The 3 patients received chemotherapy. One died of early AITL relapse. The other 2 remained in complete remission of AITL, 1 died with recurrent CLL, and 1 of acute myeloid leukemia. These observations expand the spectrum of T-cell lymphoma entities that occur in association with CLL/SLL, adding AITL to the rare variants of aggressive neoplasms manifesting as Richter syndrome. Given that disturbances of T-cell homeostasis in CLL/SLL affect not only cytotoxic but also helper T-cell subsets, these may contribute to the emergence of neoplasms of T follicular helper derivation. Abstract : Supplemental Digital Content is available in the text.