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Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia

Myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML) are associated with systemic inflammatory or autoimmune diseases in 10-20% of cases. Immune thrombocytopenia (ITP) is among the reportedly associated diseases, but large studies assessing the association are lacking. It is un...

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Autores principales: Jachiet, Vincent, Moulis, Guillaume, Hadjadj, Jérome, Seguier, Julie, Laribi, Kamel, Schleinitz, Nicolas, Vey, Norbert, Sacre, Karim, Godeau, Bertrand, Beyne-Rauzy, Odile, Bouvet, Romain, Broner, Jonathan, Brun, Natacha, Comont, Thibault, Gaudin, Clément, Lambotte, Olivier, Le Clech, Lenaïg, Peterlin, Pierre, Roy-Peaud, Frédérique, Salvado, Clémentine, Versini, Mathilde, Isnard, Françoise, Kahn, Jean Emmanuel, Gobert, Delphine, Adès, Lionel, Fenaux, Pierre, Fain, Olivier, Mekinian, Arsène
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Fondazione Ferrata Storti 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8094121/
https://www.ncbi.nlm.nih.gov/pubmed/33626866
http://dx.doi.org/10.3324/haematol.2020.272559
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author Jachiet, Vincent
Moulis, Guillaume
Hadjadj, Jérome
Seguier, Julie
Laribi, Kamel
Schleinitz, Nicolas
Vey, Norbert
Sacre, Karim
Godeau, Bertrand
Beyne-Rauzy, Odile
Bouvet, Romain
Broner, Jonathan
Brun, Natacha
Comont, Thibault
Gaudin, Clément
Lambotte, Olivier
Le Clech, Lenaïg
Peterlin, Pierre
Roy-Peaud, Frédérique
Salvado, Clémentine
Versini, Mathilde
Isnard, Françoise
Kahn, Jean Emmanuel
Gobert, Delphine
Adès, Lionel
Fenaux, Pierre
Fain, Olivier
Mekinian, Arsène
author_facet Jachiet, Vincent
Moulis, Guillaume
Hadjadj, Jérome
Seguier, Julie
Laribi, Kamel
Schleinitz, Nicolas
Vey, Norbert
Sacre, Karim
Godeau, Bertrand
Beyne-Rauzy, Odile
Bouvet, Romain
Broner, Jonathan
Brun, Natacha
Comont, Thibault
Gaudin, Clément
Lambotte, Olivier
Le Clech, Lenaïg
Peterlin, Pierre
Roy-Peaud, Frédérique
Salvado, Clémentine
Versini, Mathilde
Isnard, Françoise
Kahn, Jean Emmanuel
Gobert, Delphine
Adès, Lionel
Fenaux, Pierre
Fain, Olivier
Mekinian, Arsène
author_sort Jachiet, Vincent
collection PubMed
description Myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML) are associated with systemic inflammatory or autoimmune diseases in 10-20% of cases. Immune thrombocytopenia (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 similar platelet counts at diagnosis. First-line treatment consisted of glucocorticoids (98%) and intravenous immunoglobulins (56%). Patients with primary ITP were more likely to respond to intravenous immunoglobulins than were patients with MDS/CMML-associated ITP. Response rates to second-line therapies were not statistically different between patients with primary ITP or MDS/CMML-associated ITP. Four (10%) of the patients with MDS/CMML-associated ITP had multirefractory ITP whereas none of the primary ITP controls did so. After a median follow-up of 60 months, there was no difference in overall survival between patients with MDS/CMML-associated ITP or primary ITP. Leukemia-free-survival was significantly better in patients with MDS/CMML-associated ITP than in those with MDS/CMML without ITP. In conclusion, it appears that patients with MDS/CMML-associated ITP have a particular phenotype, with more severe bleeding than patients with primary ITP, a higher likelihood of multirefractory disease, but a similar response to primary ITP therapy except for intravenous immunoglobulins. Finally, compared to MDS/CMML patients without ITP, they are less likely to progress to having acute myeloid leukemia.
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spelling pubmed-80941212021-05-06 Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia Jachiet, Vincent Moulis, Guillaume Hadjadj, Jérome Seguier, Julie Laribi, Kamel Schleinitz, Nicolas Vey, Norbert Sacre, Karim Godeau, Bertrand Beyne-Rauzy, Odile Bouvet, Romain Broner, Jonathan Brun, Natacha Comont, Thibault Gaudin, Clément Lambotte, Olivier Le Clech, Lenaïg Peterlin, Pierre Roy-Peaud, Frédérique Salvado, Clémentine Versini, Mathilde Isnard, Françoise Kahn, Jean Emmanuel Gobert, Delphine Adès, Lionel Fenaux, Pierre Fain, Olivier Mekinian, Arsène Haematologica Article Myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML) are associated with systemic inflammatory or autoimmune diseases in 10-20% of cases. Immune thrombocytopenia (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 similar platelet counts at diagnosis. First-line treatment consisted of glucocorticoids (98%) and intravenous immunoglobulins (56%). Patients with primary ITP were more likely to respond to intravenous immunoglobulins than were patients with MDS/CMML-associated ITP. Response rates to second-line therapies were not statistically different between patients with primary ITP or MDS/CMML-associated ITP. Four (10%) of the patients with MDS/CMML-associated ITP had multirefractory ITP whereas none of the primary ITP controls did so. After a median follow-up of 60 months, there was no difference in overall survival between patients with MDS/CMML-associated ITP or primary ITP. Leukemia-free-survival was significantly better in patients with MDS/CMML-associated ITP than in those with MDS/CMML without ITP. In conclusion, it appears that patients with MDS/CMML-associated ITP have a particular phenotype, with more severe bleeding than patients with primary ITP, a higher likelihood of multirefractory disease, but a similar response to primary ITP therapy except for intravenous immunoglobulins. Finally, compared to MDS/CMML patients without ITP, they are less likely to progress to having acute myeloid leukemia. Fondazione Ferrata Storti 2021-02-25 /pmc/articles/PMC8094121/ /pubmed/33626866 http://dx.doi.org/10.3324/haematol.2020.272559 Text en Copyright© 2021 Ferrata Storti Foundation https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution Noncommercial License (by-nc 4.0) which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.
spellingShingle Article
Jachiet, Vincent
Moulis, Guillaume
Hadjadj, Jérome
Seguier, Julie
Laribi, Kamel
Schleinitz, Nicolas
Vey, Norbert
Sacre, Karim
Godeau, Bertrand
Beyne-Rauzy, Odile
Bouvet, Romain
Broner, Jonathan
Brun, Natacha
Comont, Thibault
Gaudin, Clément
Lambotte, Olivier
Le Clech, Lenaïg
Peterlin, Pierre
Roy-Peaud, Frédérique
Salvado, Clémentine
Versini, Mathilde
Isnard, Françoise
Kahn, Jean Emmanuel
Gobert, Delphine
Adès, Lionel
Fenaux, Pierre
Fain, Olivier
Mekinian, Arsène
Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia
title Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia
title_full Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia
title_fullStr Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia
title_full_unstemmed Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia
title_short Clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia
title_sort clinical spectrum, outcome and management of immune thrombocytopenia associated with myelodysplastic syndromes and chronic myelomonocytic leukemia
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8094121/
https://www.ncbi.nlm.nih.gov/pubmed/33626866
http://dx.doi.org/10.3324/haematol.2020.272559
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