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Clinical spectrum, evolution, and management of autoimmune cytopenias associated with angioimmunoblastic T‐cell lymphoma
Author(s) -
Crickx Etienne,
Poullot Elsa,
Moulis Guillaume,
Goulabchand Radjiv,
Fieschi Claire,
Galicier Lionel,
Meignin Véronique,
Coppo Paul,
Delarue Richard,
Casasnovas Olivier,
RoosWeil Damien,
de Leval Laurence,
Parrens Marie,
Michel Marc,
Dupuis Jehan,
Le Bras Fabien,
Fataccioli Virginie,
MartinGarcia Nadine,
Godeau Bertrand,
Haïoun Corinne,
Gaulard Philippe,
Mahévas Matthieu
Publication year - 2019
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/ejh.13239
Subject(s) - angioimmunoblastic t cell lymphoma , medicine , cytopenia , lymphoma , bone marrow , autoimmune lymphoproliferative syndrome , gastroenterology , immunology , t cell , biology , immune system , apoptosis , biochemistry , fas receptor , programmed cell death
Objective Angioimmunoblastic T‐cell lymphoma (AITL) is frequently associated with autoimmune cytopenia (AIC). Whether such patients have a particular phenotype and require particular management is unclear. Method Angioimmunoblastic T‐cell lymphoma patients from the multicentric database of the Lymphoma Study Association presenting with AIC during disease course were included and matched to AITL patients without AIC (1/5 ratio). Results At diagnosis, AIC patients (n = 28) had more spleen and bone marrow involvement (54% vs 19% and 71% vs 34%, P < 0.001), Epstein‐Barr virus replication (89% vs 39%, P < 0.001), gamma globulin titers (median 23 vs 15 g/L, P = 0.002), and proliferating B cells and plasmablasts in biopsies, as compared to control patients (n = 136). The 28 AIC patients had 41 episodes of AIC, diagnosed concomitantly with AITL in 23 (82%) cases. After a median follow‐up of 24 months (range 3‐155), 10 patients relapsed, all associated with AITL relapse. Conclusion Our results provide new insight into AIC associated with AITL by highlighting the significant interplay between AITL and B‐cell activation leading to subsequent autoimmunity.