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

Details

Serval ID
serval:BIB_DC97D082A0AB
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Angioimmunoblastic T-Cell Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma: A Novel Form of Composite Lymphoma Potentially Mimicking Richter Syndrome.
Journal
The American journal of surgical pathology
Author(s)
Trimech M., Letourneau A., Missiaglia E., De Prijck B., Nagy-Hulliger M., Somja J., Vivario M., Gaulard P., Lambert F., Bisig B., de Leval L.
ISSN
1532-0979 (Electronic)
ISSN-L
0147-5185
Publication state
Published
Issued date
01/06/2021
Peer-reviewed
Oui
Volume
45
Number
6
Pages
773-786
Language
english
Notes
Publication types: Case Reports ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
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.
Keywords
Aged, Aged, 80 and over, Antineoplastic Combined Chemotherapy Protocols/therapeutic use, Biomarkers, Tumor/genetics, Composite Lymphoma/drug therapy, Composite Lymphoma/genetics, Composite Lymphoma/immunology, Diagnosis, Differential, Fatal Outcome, Female, Gene Rearrangement, Genes, T-Cell Receptor, Humans, Immunoblastic Lymphadenopathy/drug therapy, Immunoblastic Lymphadenopathy/genetics, Immunoblastic Lymphadenopathy/immunology, Immunoglobulins/genetics, Leukemia, Lymphocytic, Chronic, B-Cell/drug therapy, Leukemia, Lymphocytic, Chronic, B-Cell/genetics, Leukemia, Lymphocytic, Chronic, B-Cell/immunology, Lymphoma, T-Cell/drug therapy, Lymphoma, T-Cell/genetics, Lymphoma, T-Cell/immunology, Male, Predictive Value of Tests, Recurrence, Time Factors, Treatment Outcome
Pubmed
Web of science
Create date
22/03/2021 9:42
Last modification date
11/08/2021 6:38
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