Claire Rivoisy1, Benjamin Besse2, Nicolas Girard3, Bertrand Lioger4, Jean-Francois Viallard5, Jean-Christophe Lega6, Patricia Rullier7, Antoinette Perlat8, Delphine Lerouge9, Christelle Clement-Duchene10, Mikael Ebbo11, Annick Bosseray12, Bertrand Godeau13, Olivier Lambotte14. 1. Department of Internal Medicine, Bicêtre Hospital, Le Kremlin Bicêtre, France. 2. Department of Cancer Medicine, Gustave Roussy Institute, Villejuif, France Paris-Sud University, Paris, France. 3. Department of Respiratory Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France. 4. Department of Internal Medicine, University Medical Center Tours, Tours, France. 5. Internal Medicine Department, Bordeaux University Medical Center, Pessac, France. 6. UMR, Unite Mixte de Recherche 5558, Department of Internal and Vascular Medicine, University Medical Center Lyon Sud, Claude Bernard University Lyon 1, France; Biometry and Evolutionary Biology Laboratory, Centre National de la Recherche Scientifique, Claude Bernard University Lyon 1, France. 7. Department of Internal Medicine, University Medical Center Montpellier, Montpellier, France. 8. Department of Internal Medicine, University Medical Center Rennes, Rennes, France. 9. Deparment of Pneumology and Thoracic Oncology, University Medical Center Caen, France. 10. Chest Department, University Medical Center Nancy, Nancy, France; Research Team EA, Equipe Avenir 7298, INGRES, Lorraine University, Lorraine, France. 11. Department of Internal Medicine, La Conception Hospital, University Medical Center Marseille, Aix-Marseille University, Marseille, France. 12. Department of Internal Medicine, University Medical Center Grenoble, Grenoble, France. 13. Department of Internal Medicine, National Reference Centre for Autoimmune Cytopenia in Adults, Henri-Mondor University Hospital, University Medical Center Paris, Paris-Est Créteil University, Créteil, France. 14. Department of Internal Medicine, Bicêtre Hospital, Le Kremlin Bicêtre, France; Paris Sud University, UMR, Unite Mixte de Recherche 1184, France; CEA, DSV/iMETI, Division of Immuno-Virology, Infectious Disease Models and Innovative Therapies, Le Kremlin Bicêtre, France; Inserm, U1184, Center for Immunology of Viral Infections and Autoimmune Diseases, Le Kremlin Bicêtre, France. Electronic address: olivier.lambotte@bct.aphp.fr.
Abstract
INTRODUCTION: Thymic epithelial tumor (TET)-associated cytopenia is rare but difficult to treat. METHODS: We performed a multicenter, retrospective study of TET and associated forms of cytopenia in France. Cases were collected by the French National Reference Center for Autoimmune Cytopenia and the French National Thymic Malignancy Interest Group (Réseau Tumeurs Thymiques et Cancer) and through a call for cases by the French Society of Internal Medicine. RESULTS: Thirty-six cases were recorded between 2002 and 2014 and followed up for a median of 38 months (interquartile range, 23-106 months). Thirty-two patients underwent surgery for TET, and 14 of the latter were in complete remission at last follow-up. Cytopenia can occur before, simultaneously, or after diagnosis of TET. The most common types of cytopenia were pure red cell aplasia (in 30% of cases) and Good syndrome (GS) (also in 30% of cases). Eleven patients displayed two or more episodes of cytopenia. Eighteen patients received steroids as their first-line treatment, leading to a complete response in nine. Other first-line treatments (cyclosporine and rituximab) were less effective but should be considered as treatment options. Infections developed in 84% of the patients with GS; this did not appear to be related to the presence or absence of immunosuppressive treatment or chemotherapy. Eight patients died during the follow-up period (two died of cytopenia and five of infections). CONCLUSIONS: The optimal treatment for TET-associated cytopenia has not been clearly defined and the outcome does not appear to be correlated with TET progression. For GS, prophylactic immunoglobulin replacement therapy and prophylactic antibiotic therapy can be recommended.
INTRODUCTION: Thymic epithelial tumor (TET)-associated cytopenia is rare but difficult to treat. METHODS: We performed a multicenter, retrospective study of TET and associated forms of cytopenia in France. Cases were collected by the French National Reference Center for Autoimmune Cytopenia and the French National Thymic Malignancy Interest Group (Réseau Tumeurs Thymiques et Cancer) and through a call for cases by the French Society of Internal Medicine. RESULTS: Thirty-six cases were recorded between 2002 and 2014 and followed up for a median of 38 months (interquartile range, 23-106 months). Thirty-two patients underwent surgery for TET, and 14 of the latter were in complete remission at last follow-up. Cytopenia can occur before, simultaneously, or after diagnosis of TET. The most common types of cytopenia were pure red cell aplasia (in 30% of cases) and Good syndrome (GS) (also in 30% of cases). Eleven patients displayed two or more episodes of cytopenia. Eighteen patients received steroids as their first-line treatment, leading to a complete response in nine. Other first-line treatments (cyclosporine and rituximab) were less effective but should be considered as treatment options. Infections developed in 84% of the patients with GS; this did not appear to be related to the presence or absence of immunosuppressive treatment or chemotherapy. Eight patients died during the follow-up period (two died of cytopenia and five of infections). CONCLUSIONS: The optimal treatment for TET-associated cytopenia has not been clearly defined and the outcome does not appear to be correlated with TET progression. For GS, prophylactic immunoglobulin replacement therapy and prophylactic antibiotic therapy can be recommended.
Authors: Anna Maria Malfitano; Vittoria D'Esposito; Pietro De Placido; Marianna Tortora; Margaret Ottaviano; Erica Pietroluongo; Rocco Morra; Brigitta Mucci; Fabiana Napolitano; Liliana Montella; Mario Giuliano; Sabino De Placido; Daniela Terracciano; Giovannella Palmieri; Pietro Formisano Journal: Front Immunol Date: 2022-08-18 Impact factor: 8.786
Authors: Sofia Chiatamone Ranieri; Stefania Trasarti; Maria Antonietta Arleo; Luisa Bizzoni; Livia Bonanni; Valeria Di Battista; Maria Assunta Limongiello; Maria Grazia Nardacci; Giuseppe Gentile; Robin Foà Journal: Case Rep Hematol Date: 2019-11-12