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Ferrata Storti Foundation
Platelet Biology & its Disorders
Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia
Vincent Jachiet,1 Guillaume Moulis,2 Jérome Hadjadj,3 Julie Seguier,4 Kamel Laribi,5 Nicolas Schleinitz,4 Norbert Vey,6 Karim Sacre,7 Bertrand Godeau,8 Odile Beyne-Rauzy,9 Romain Bouvet,10 Jonathan Broner,11 Natacha Brun,12 Thibault Comont,9 Clément Gaudin,13 Olivier Lambotte,14 Lenaïg Le Clech,15 Pierre Peterlin,16 Frédérique Roy-Peaud,17 Clémentine Salvado,18 Mathilde Versini,19 Françoise Isnard,20 Jean Emmanuel Kahn,21 Delphine Gobert,1 Lionel Adès,22 Pierre Fenaux,22 Olivier Fain1 and Arsène Mekinian1, on behalf of MIN- HEMON, GFM, CERECAI and CARMEN investigators group*
Haematologica 2021 Volume 106(5):1414-1422
1Service de Médecine Interne and Inflammation-Immunopathology-Biotherapy Department (DMU 3iD), Hôpital Saint-Antoine, Assistance Publique – Hôpitaux de Paris, Sorbonne Université, Paris; 2Service de Médecine Interne, Centre Hospitalier Universitaire de Toulouse, Université de Toulouse, Toulouse; 3Department of Internal Medicine, Hôpital Cochin, Assistance Publique – Hôpitaux de Paris, Université de Paris, Paris; 4Département de Médecine Interne, Hôpital de la Timone, Assistance Publique – Hôpitaux de Marseille, Aix Marseille Université, Marseille; 5Department of Hematology, Centre Hospitalier Le Mans, Le Mans; 6Hematology Department, Institut Paoli- Calmettes, Aix-Marseille Université, Marseille; 7Departement de Médecine Interne, Hôpital Bichat, Assistance Publique – Hôpitaux de Paris, Université de Paris, Paris; 8Médecine Interne, Hôpital Henri Mondor, Assistance Publique – Hôpitaux de Paris, Université Paris-Est Créteil, Créteil; 9Department of Internal Medicine, Institut Universitaire du Cancer de Toulouse-Oncopole, Toulouse; 10Médecine Interne et Maladies Systémiques, Centre Hospitalier Universitaire Dijon Bourgogne, Dijon; 11Internal Médicine Department, Nîmes University Hospital, University of Montpellier, Nîmes; 12Service de Médecine Interne, Centre Hospitalier de Rodez, Rodez; 13Department of Oncogeriatric Medicine, University Hospital Purpan, Toulouse; 14Médecine Interne et Immunologie Clinique, Hôpital Bicêtre, Assistance Publique – Hôpitaux de Paris, Université Paris Sud, Le Kremlin-Bicêtre; 15Department of Internal Medicine, Infectious Diseases and Hematology, Cornouaille Hospital Quimper, Quimper; 16Service d'Hématologie Clinique, Centre Hospitalier Universitaire de Nantes, Nantes; 17Service de Médecine Interne, Maladies Infectieuses et Tropicales, Centre Hospitalier Universitaire de Poitiers, Poitiers; 18Service de Maladies Infectieuses, Centre Hospitalier de Dax, Dax; 19Institut Arnault Tzanck, Saint Laurent du Var; 20Department of Clinical Hematology, Hôpital Saint-Antoine, Assistance Publique – Hôpitaux de Paris, Sorbonne Université, Paris; 21Service de Médecine Interne, Hôpital Ambroise Paré, Assistance Publique – Hôpitaux de Paris, Paris and 22Service d'Hématologie Clinique, Department of Hematology, Hôpital St. Louis, Assistance Publique – Hôpitaux de Paris, Université de Paris, Paris, France
* Listed in the Appendix
ABSTRACT
Myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML) are associated with systemic inflammatory or autoimmune diseases in 10-20% of cases. Immune thrombocy- topenia (ITP) is among the reportedly associated diseases, but large studies assessing the association are lacking. It is unclear whether patients with MDS or CMML and ITP have a particular phenotype or require particular management. We, therefore, analyzed the clinical spectrum, outcome and therapeutic management of patients with ITP associated with MDS or CMML, in comparison to: (i) patients with primary ITP without MDS/CMML and (ii) patients with MDS/CMML without ITP. Forty-one patients with MDS/CMML-associated ITP were included, of whom 26 (63%) had chronic ITP, 30 (73%) had low-risk myelodysplasia and 24 (59%) had CMML. An associated autoimmune disease was noted in ten (24%) patients. In comparison to patients with primary ITP, patients with MDS/CMML-associated ITP had a higher rate of severe bleeding despite
Correspondence:
VINCENT JACHIET
vincent.jachiet@aphp.fr
Received: September 17, 2020. Accepted: November 23, 2020. Pre-published: February 25, 2021.
https://doi.org/10.3324/haematol.2020.272559
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