Angioimmunoblastic T-Cell Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma: A Novel Form of Composite Lymphoma Potentially Mimicking Richter Syndrome.
[en] 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.
Disciplines :
Hematology
Author, co-author :
Trimech, Mounir; Department of Laboratory Medicine and Pathology, Institute of Pathology, Lausanne University Hospital and Lausanne University, Lausanne
Letourneau, Audrey; Department of Laboratory Medicine and Pathology, Institute of Pathology, Lausanne University Hospital and Lausanne University, Lausanne
Missiaglia, Edoardo; Department of Laboratory Medicine and Pathology, Institute of Pathology, Lausanne University Hospital and Lausanne University, Lausanne
DE PRIJCK, Bernard ; Centre Hospitalier Universitaire de Liège - CHU > > Service d'hématologie clinique ; Department of Medicine, Division of Hematology
Nagy-Hulliger, Monika; Service of Hematology, Hospital of Morges, Morges, Switzerland
SOMJA, Joan ; Centre Hospitalier Universitaire de Liège - CHU > > Service dermatopathologie
Vivario, Manuela; Department of Pathology, University Hospital of Liège
Gaulard, Philippe; Department of Pathology, Henri Mondor Hospital, AP-HP ; INSERM U955, Mondor Institute for Biomedical Research (IMRB ; Paris Est Creteil University, Créteil, France
LAMBERT, Frédéric ; Centre Hospitalier Universitaire de Liège - CHU > > Service de génétique
Bisig, Bettina; Department of Laboratory Medicine and Pathology, Institute of Pathology, Lausanne University Hospital and Lausanne University, Lausanne
de Leval, Laurence; Department of Laboratory Medicine and Pathology, Institute of Pathology, Lausanne University Hospital and Lausanne University, Lausanne
Language :
English
Title :
Angioimmunoblastic T-Cell Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma: A Novel Form of Composite Lymphoma Potentially Mimicking Richter Syndrome.
Publication date :
2021
Journal title :
American Journal of Surgical Pathology
ISSN :
0147-5185
eISSN :
1532-0979
Publisher :
Lippincott Williams and Wilkins, United States
Volume :
45
Pages :
773-786
Peer reviewed :
Peer Reviewed verified by ORBi
Funding text :
Conflicts of Interest and Source of Funding: Supported by a grant of the National Swiss Foundation. The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.
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