Characteristics of thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly syndrome: a retrospective study from a large Western cohort

Autor: Margaux Garzaro, David Boutboul, B. Dunogué, Rémi Bertinchamp, Lionel Galicier, Frédéric Vandergeynst, Mirlinda Berisha, Maximilien Grall, Virginie De Wilde, Noémie Saada, Eric Oksenhendler, Jehane Fadlallah, Laurence Gérard, Lucas Maisonobe, Stephanie Guillet, M. Hie, Marion Malphettes, Lara Zafrani, Claire Fieschi, Louise Damian
Přispěvatelé: Immunologie clinique [CHU St-Louis], Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de médecine interne [Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Service de Médecine Interne 2, maladies auto-immunes et systémiques [CHU Pitié-Salpêtrière], Institut E3M [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Erasme [Bruxelles] (ULB), Faculté de Médecine [Bruxelles] (ULB), Université libre de Bruxelles (ULB)-Université libre de Bruxelles (ULB), Service d'Anesthésie-Réanimation [AP-HP Hôpitaux Saint-Louis Lariboisière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Cité (UPCité), Service de Médecine Interne [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), CHU Henri Mondor, Immunologie humaine, physiopathologie & immunothérapie (HIPI (UMR_S_976 / U976)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Gestionnaire, Hal Sorbonne Université
Jazyk: angličtina
Rok vydání: 2022
Předmět:
[SDV.MHEP.HEM] Life Sciences [q-bio]/Human health and pathology/Hematology
Adult
Male
Pediatrics
medicine.medical_specialty
Biopsy
Clinical Decision-Making
Anasarca
Organomegaly
anasarca
Diagnosis
Differential
chemistry.chemical_compound
Young Adult
tocilizumab
Tocilizumab
rituximab
Positron Emission Tomography Computed Tomography
Medicine
Humans
Retrospective Studies
fever
business.industry
urogenital system
Castleman Disease
Disease Management
Retrospective cohort study
[SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology
Hematology
Syndrome
Middle Aged
Prognosis
Combined Modality Therapy
Immunohistochemistry
Phenotype
Treatment Outcome
chemistry
Reticulin fibrosis
idiopathic multicentric Castleman disease
Cohort
Inclusion and exclusion criteria
Western thrombocytopenia
Rituximab
Female
Disease Susceptibility
medicine.symptom
business
medicine.drug
reticulin fibrosis and organomegaly syndrome
Zdroj: British Journal of Haematology
British Journal of Haematology, Wiley, In press, ⟨10.1111/bjh.17868⟩
ISSN: 0007-1048
1365-2141
DOI: 10.1111/bjh.17868⟩
Popis: International audience; Idiopathic multicentric Castleman disease (iMCD) is a non-clonal inflammatory lymphoproliferative disorder of unknown origin. Recently, TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly) emerged as a singular variant of iMCD in Asia and was associated with a severe course and a poor outcome. The present study describes the first large Western cohort of TAFRO syndrome patients (n = 25) meeting the All Japan TAFRO Syndrome Research Group diagnostic criteria. Characteristics of TAFRO patients were compared to iMCD-not otherwise specified (iMCD-NOS) patients used as a control group (n = 43). Our results show that despite baseline characteristics in accordance with previously reported series, Western TAFRO syndrome patients do not appear to present with a worse outcome than iMCD-NOS patients. There were no significant differences between the two groups regarding treatment choice, response to rituximab (71% vs. 67%) or tocilizumab (69% vs. 91%) in TAFRO and iMCD-NOS, respectively. The two-year overall survival was above 95% in both groups. Limits of inclusion and exclusion criteria for TAFRO definition are also discussed. Our findings raise the question of the singularity of the TAFRO entity in Western countries. The data should promote further research using unsupervised models to identify markers of disease severity in Western cohorts of iMCD patients.
Databáze: OpenAIRE
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