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Letters to the Editor
criterion allowed us to objectively assess response to immunosuppressive therapy but requires further adapta- tion in the context of an AID to be fully relevant. Nevertheless, most cases (27 of 29 treated patients) received GC alone or combined with other immunosup- pressive agents as first-line therapy, which allowed for CR or CR lacking CBMB in more than 60% of cases. These findings seem to suggest that GC remain the first- choice therapy because of response in more than 50% of cases, but a high rate of GC dependency and long-term complications indicate a need to find new sparing drugs.
In case of persistent neutropenia and anemia, no infec- tious complications seemed to occur and no transfusion dependency was observed, so treatment escalation with additional immunomodulatory or immunosuppressive agents may not be indicated in these cases. In contrast, persistent thrombocytopenia might indicate a specific treatment because of persistent risk of hemorrhagic syn- drome.
In conclusion, this analysis of 30 unique French cases of AIMF is the largest to date. These findings may help improve early diagnosis of this rare disease and allow for improvement and homogenization of the set of therapeu- tic tools to be used in future studies.
Philippe Mertz,1-2 Emilie Chalayer,3,4 Zahir Amoura,5 Pascal Cathebras,6 Laurent Chiche,7 Nathalie Costedoat,8 Alban Deroux,9 Elisabeth Diot,10 Stéphane Durupt,11 Emmanuel Forestier,12 Audrey Gorse,12 Laurent Hudier,13 Martin Killian,6 Olivier Lambotte,14,15 Claire Lecomte,16 Emmanuel Ledoult,17 Camille Martinez,18 Alexis Mathian,5 Anne-Sophie Morin,19 Nicolas Noël,14,15
Marc Pineton De Chambrun,5 Louis Terriou,17 Jean Sibilia,1 Jacques-Eric Gottenberg,1 Anne-Sophie Korganow,2
Laurent Arnaud1 and Thierry Martin2
1Service de Rhumatologie, Hôpitaux Universitaires de Strasbourg, Laboratoire d'ImmunoRhumatologie Moléculaire, INSERM UMR_S1109, Centre National de Référence des Maladies Systémiques et Autoimmunes Rares Est Sud-Ouest (RESO), Université de Strasbourg, Strasbourg; 2Service de Médecine Interne et d'Immunologie Clinique, Hôpitaux Universitaires de Strasbourg, Centre National de Référence des Maladies Systémiques et Autoimmunes Rares Est Sud-Ouest (RESO), Université de Strasbourg, Strasbourg; 3Institut de Cancérologie Lucien Neuwirth, Saint Priest en Jarez; 4Dysfonction Vasculaire et Hémostase, INSERM, SAINBIOSE, Université Jean Monnet, Saint-Etienne; 5French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, INSERM, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Groupement Hospitalier Pitié-Salpêtrière, Paris; 6Service de Médecine Interne, CHU de Saint-Étienne, Hôpital Nord, Saint-Étienne; 7Unité de Soins et de Recherche en Médecine Interne, Hôpital Européen, Marseille; 8AP-HP, Cochin Hospital, Dpeartment of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Paris; Université de Paris, CRESS, INSERM, INRA, Paris; 9Service de Médecine Interne, Centre Hospitalier Universitaire Grenoble, Michallon Hospital, Grenoble; 10Internal Medicine, CHU Tours, University of Tours, Tours; 11Service de Médecine Interne et de Pathologie Vasculaire, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite; 12Service de Maladies Infectieuses et Médecine Interne, Centre Hospitalier Métropole Savoie, Chambéry; 13Centre Hospitalier Broussais, Service
de Néphrologie-Hémodialyse, Saint-Malo; 14Service de Médecine Interne et Immunologie Clinique, APHP CHU Bicêtre, Le Kremlin Bicêtre; 15Université Paris-Sud, Center for Immunology of Viral Infections and Auto-Immune Diseases (IMVA), Institut pour la Santé et la Recherche Médicale INSERM UMR 1184, Université Paris- Sud, Le Kremlin-Bicêtre; 16Service de Médecine Interne et Maladies Infectieuses, Hôpital Mutualiste, Villeurbanne; 17Département de Médecine Interne et Immunologie Clinique, CHU Lille, Centre de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Institute for Translational Research in Inflammation (INFINITE), INSERM U1286, Université de Lille, Lille; 18Service de Médecine Interne, CHR Colmar and 19Service de Médecine Interne, Hôpital Jean Verdier, AP-HP, Bondy, France
Correspondence:
THIERRY MARTIN - thierry.martin@chru-strasbourg.fr
doi:10.3324/haematol.2020.249896
Disclosures: no conflicts of interest to disclose.
Contributions: all authors were involved in drafting the article or revising it critically for important intellectual content, and all authors approved the final version to be published. Study conception and design: PM, LA, TM; acquisition of data: PM, EC, ZA, PC, LC, NC-C, AD, SD, EF, AG, LH, MK, OL, EL, CM, AM, A-SM, NN, MPDC, LT, JS, J-EG, A-SSK, LA, TM; analysis and interpretation of data: PM, EC, ZA, PC, LC, NC-C, AD, SD, EF, AG, LH, MK, OL, EL, CM, AM, A-SM, NN, MPDC, LT, JS, J-EG, A-SK, LA, TM.
References
1. Pullarkat V, Bass RD, Gong JZ, Feinstein DI, Brynes RK. Primary autoimmune myelofibrosis: definition of a distinct clinicopathologic syndrome. Am J Hematol. 2003;72(1):8-12.
2. Paquette RL, Meshkinpour A, Rosen PJ. Autoimmune myelofibro- sis. A steroid-responsive cause of bone marrow fibrosis associated with systemic lupus erythematosus. Medicine (Baltimore). 1994; 73(3):145-152.
3. Tefferi A, Cervantes F, Mesa R, et al. Revised response criteria for myelofibrosis: International Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) and European LeukemiaNet (ELN) consensus report. Blood. 2013;122(8):1395- 1398.
4. Barcellini W, Iurlo A, Radice T, et al. Increased prevalence of autoim- mune phenomena in myelofibrosis: relationship with clinical and morphological characteristics, and with immunoregulatory cytokine patterns. Leuk Res. 2013;37(11):1509-1515.
5. Tan EM, Feltkamp TE, Smolen JS, et al. Range of antinuclear anti- bodies in “healthy” individuals. Arthritis Rheum. 1997;40(9):1601- 1611.
6. Kristinsson SY, Landgren O, Samuelsson J, Björkholm M, Goldin LR. Autoimmunity and the risk of myeloproliferative neoplasms. Haematologica. 2010;95(7):1216-1220.
7. Marcellino B, El Jamal SM, Mascarenhas JO. Distinguishing autoim- mune myelofibrosis from primary myelofibrosis. Clin Adv Hematol Oncol 2018;16(9):619–626.
8. Vergara-Lluri ME, Piatek CI, Pullarkat V, et al. Autoimmune myelofi- brosis: an update on morphologic features in 29 cases and review of the literature. Hum Pathol. 2014;45(11):2183-2191.
9. Zahr AA, Salama ME, Carreau N, et al. Bone marrow fibrosis in myelofibrosis: pathogenesis, prognosis and targeted strategies. Haematologica. 2016;101(6):660-671.
10. Tefferi A, Lasho TL, Jimma T, et al. One thousand patients with pri- mary myelofibrosis: the mayo clinic experience. Mayo Clin Proc. 2012;87(1):25-33.
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