title: Thymic Epithelial Tumor-Associated Cytopenia: A 10-Year Observational Study in France.
abstract: BACKGROUND
  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.affiliation: Department of Internal Medicine, Bicêtre Hospital, Le Kremlin Bicêtre, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Rivoisy
authors.affiliation: Department of Cancer Medicine, Gustave Roussy Institute, Villejuif, France Paris-Sud University, Paris, France.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Besse
authors.affiliation: Department of Respiratory Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Girard
authors.affiliation: Department of Internal Medicine, University Medical Center Tours, Tours, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Lioger
authors.affiliation: Internal Medicine Department, Bordeaux University Medical Center, Pessac, France.
authors.forename: Jean-Francois
authors.initials: JF
authors.lastname: Viallard
authors.affiliation: 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.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Lega
authors.affiliation: Department of Internal Medicine, University Medical Center Montpellier, Montpellier, France.
authors.forename: Patricia
authors.initials: P
authors.lastname: Rullier
authors.affiliation: Department of Internal Medicine, University Medical Center Rennes, Rennes, France.
authors.forename: Antoinette
authors.initials: A
authors.lastname: Perlat
authors.affiliation: Deparment of Pneumology and Thoracic Oncology, University Medical Center Caen, France.
authors.forename: Delphine
authors.initials: D
authors.lastname: Lerouge
authors.affiliation: Chest Department, University Medical Center Nancy, Nancy, France; Research Team EA, Equipe Avenir 7298, INGRES, Lorraine University, Lorraine, France.
authors.forename: Christelle
authors.initials: C
authors.lastname: Clement-Duchene
authors.affiliation: Department of Internal Medicine, La Conception Hospital, University Medical Center Marseille, Aix-Marseille University, Marseille, France.
authors.forename: Mikael
authors.initials: M
authors.lastname: Ebbo
authors.affiliation: Department of Internal Medicine, University Medical Center Grenoble, Grenoble, France.
authors.forename: Annick
authors.initials: A
authors.lastname: Bosseray
authors.affiliation: 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.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.affiliation: 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.
authors.forename: Olivier
authors.initials: O
authors.lastname: Lambotte
mesh_terms: D000368:Aged; D005602:France; D006801:Humans; D008297:Male; D008875:Middle Aged; D009375:Neoplasms, Glandular and Epithelial / Q000097:blood* / Q000473:pathology; D012189:Retrospective Studies; D013921:Thrombocytopenia / Q000473:pathology*; D013953:Thymus Neoplasms / Q000097:blood* / Q000473:pathology
keywords: Good syndrome; Infectious complications; Pure red cell aplasia; Thymic epithelial tumor