Étude du profil lymphocytaire en situation de rechute précoce et tardive après allogreffe de cellules souches hématopoïétiques

Le risque de rechute post-allogreffe de moelle dans un contexte de leucémie myéloïde aigue (AML) varie entre 35 et 45% selon différentes variables considérées, et dépasse 50% dans le cas des syndromes myélodysplasiques (MDS). Une immunité anti-tumorale défective, au coeur d’interactions immunologiques complexes, en constitue l’une des causes majeures. Nous nous sommes intéressés au profil lymphocytaire de patients ayant rechuté après allogreffe de moelle dans un contexte d’AML ou de MDS ainsi qu’à des variables cliniques précises afin d’établir d’éventuelles corrélations clinico-biologiques. Cette analyse fait suite au protocole « DLI-Vidaza » ayant testé l’efficacité du Vidaza + DLI chez des patients rechuteurs, dont elle constitue en partie l’objectif tertiaire

Complete Remission in a TEMPI Syndrome Treated with a Daratumumab, Lenalidomide, and Dexamethasone-Based Regimen: A Case Report.

TEMPI syndrome is a rare and acquired condition which is characterized by five classical features: telangiectasias, erythrocytosis with elevated erythropoietin, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting. The classical treatment is based on bortezomib which can achieve variable responses. Relapse or refractory disease may occur, so other treatment strategies can be proposed. We describe the case of a 54-year-old male followed for a refractory TEMPI syndrome who achieved complete remission after a second-line therapy composed of daratumumab-, lenalidomide-, and dexamethasone-based regimen (DLd). He achieved a complete remission with dramatic improvement of his renal function, restitution of a normal blood oxygen, and disappearance of polycythemia. This case highlights the effectiveness of an association of DLd to treat refractory TEMPI syndrome. We also provide arguments for an association between TEMPI syndrome and monoclonal gammopathy of renal significance

Complete Remission in a TEMPI Syndrome Treated with a Daratumumab, Lenalidomide, and Dexamethasone-Based Regimen: A Case Report

Introduction: TEMPI syndrome is a rare and acquired condition which is characterized by five classical features: telangiectasias, erythrocytosis with elevated erythropoietin, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting. The classical treatment is based on bortezomib which can achieve variable responses. Relapse or refractory disease may occur, so other treatment strategies can be proposed. Case Presentation: We describe the case of a 54-year-old male followed for a refractory TEMPI syndrome who achieved complete remission after a second-line therapy composed of daratumumab-, lenalidomide-, and dexamethasone-based regimen (DLd). He achieved a complete remission with dramatic improvement of his renal function, restitution of a normal blood oxygen, and disappearance of polycythemia. Conclusion: This case highlights the effectiveness of an association of DLd to treat refractory TEMPI syndrome. We also provide arguments for an association between TEMPI syndrome and monoclonal gammopathy of renal significance