Cargando…
A French National Survey on Clotting Disorders in Mastocytosis
Mastocytosis is characterized by a clonal mast cell proliferation with organ infiltration and uncontrolled degranulation. Although not characteristic and poorly explained, some patients develop clotting abnormalities. We retrospectively identified patients with established diagnosis of mastocytosis...
Autores principales: | , , , , , , , , , , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer Health
2015
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4616764/ https://www.ncbi.nlm.nih.gov/pubmed/26447996 http://dx.doi.org/10.1097/MD.0000000000001414 |
_version_ | 1782396707448815616 |
---|---|
author | Carvalhosa, Ana B. Aouba, Achille Damaj, Gandhi Canioni, Danielle Brouzes, Chantal Gyan, Emmanuel Durupt, Stéphane Durieu, Isabelle Cathebras, Pascal Costédoat-Chalumeau, Nathalie Launay, David Pilmis, Benoit Barete, Stephane Frenzel, Laurent Lortholary, Olivier Hermine, Olivier Hermans, Cedric Chandesris, Marie-Olivia |
author_facet | Carvalhosa, Ana B. Aouba, Achille Damaj, Gandhi Canioni, Danielle Brouzes, Chantal Gyan, Emmanuel Durupt, Stéphane Durieu, Isabelle Cathebras, Pascal Costédoat-Chalumeau, Nathalie Launay, David Pilmis, Benoit Barete, Stephane Frenzel, Laurent Lortholary, Olivier Hermine, Olivier Hermans, Cedric Chandesris, Marie-Olivia |
author_sort | Carvalhosa, Ana B. |
collection | PubMed |
description | Mastocytosis is characterized by a clonal mast cell proliferation with organ infiltration and uncontrolled degranulation. Although not characteristic and poorly explained, some patients develop clotting abnormalities. We retrospectively identified patients with established diagnosis of mastocytosis and related clotting abnormalities (clinical and/or biological) using the national French Reference Centre for Mastocytosis database. From our cohort of 14 adult patients with clotting abnormalities (median age 46 years [range 26–75]), 4 had a presentation suggestive of a primary hemostasis disorder alone (by their symptoms and/or abnormal clotting tests [PFA, von Willebrand's disease [vWD] screening]) and 10 had a laboratory impairment of secondary hemostasis. Among these, 7 had bleeds characteristic of a coagulation cascade disorder (severe/life-threatening in 5 and mild in 2 patients). Clotting abnormalities were of variable severity, typically related to intense crisis of degranulation, such as anaphylactic reactions, and/or to severe organ infiltration by mast cells. Importantly, classical hemostatic management with platelet transfusion, fresh frozen plasma, or vitamin K infusions was unsuccessful, as opposed to the use of agents inhibiting mast cell activity, particularly steroids. This illustrates the crucial role of mast cell mediators such as tryptase and heparin, which interfere both with primary (mainly via inhibition of von Willebrand factor) and secondary hemostasis. There was interestingly an unusually high number of aggressive mastocytosis (particularly mast cell leukemia) and increased mortality in the group with secondary hemostasis disorders (n = 5, 36% of the whole cohort). Mast cell degranulation and/or high tumoral burden induce both specific biologic antiaggregant and anticoagulant states with a wide clinical spectrum ranging from asymptomatic to life-threatening bleeds. Hemostatic control is achieved by mast cell inhibitors such as steroids. |
format | Online Article Text |
id | pubmed-4616764 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Wolters Kluwer Health |
record_format | MEDLINE/PubMed |
spelling | pubmed-46167642015-10-27 A French National Survey on Clotting Disorders in Mastocytosis Carvalhosa, Ana B. Aouba, Achille Damaj, Gandhi Canioni, Danielle Brouzes, Chantal Gyan, Emmanuel Durupt, Stéphane Durieu, Isabelle Cathebras, Pascal Costédoat-Chalumeau, Nathalie Launay, David Pilmis, Benoit Barete, Stephane Frenzel, Laurent Lortholary, Olivier Hermine, Olivier Hermans, Cedric Chandesris, Marie-Olivia Medicine (Baltimore) 4800 Mastocytosis is characterized by a clonal mast cell proliferation with organ infiltration and uncontrolled degranulation. Although not characteristic and poorly explained, some patients develop clotting abnormalities. We retrospectively identified patients with established diagnosis of mastocytosis and related clotting abnormalities (clinical and/or biological) using the national French Reference Centre for Mastocytosis database. From our cohort of 14 adult patients with clotting abnormalities (median age 46 years [range 26–75]), 4 had a presentation suggestive of a primary hemostasis disorder alone (by their symptoms and/or abnormal clotting tests [PFA, von Willebrand's disease [vWD] screening]) and 10 had a laboratory impairment of secondary hemostasis. Among these, 7 had bleeds characteristic of a coagulation cascade disorder (severe/life-threatening in 5 and mild in 2 patients). Clotting abnormalities were of variable severity, typically related to intense crisis of degranulation, such as anaphylactic reactions, and/or to severe organ infiltration by mast cells. Importantly, classical hemostatic management with platelet transfusion, fresh frozen plasma, or vitamin K infusions was unsuccessful, as opposed to the use of agents inhibiting mast cell activity, particularly steroids. This illustrates the crucial role of mast cell mediators such as tryptase and heparin, which interfere both with primary (mainly via inhibition of von Willebrand factor) and secondary hemostasis. There was interestingly an unusually high number of aggressive mastocytosis (particularly mast cell leukemia) and increased mortality in the group with secondary hemostasis disorders (n = 5, 36% of the whole cohort). Mast cell degranulation and/or high tumoral burden induce both specific biologic antiaggregant and anticoagulant states with a wide clinical spectrum ranging from asymptomatic to life-threatening bleeds. Hemostatic control is achieved by mast cell inhibitors such as steroids. Wolters Kluwer Health 2015-10-09 /pmc/articles/PMC4616764/ /pubmed/26447996 http://dx.doi.org/10.1097/MD.0000000000001414 Text en Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 |
spellingShingle | 4800 Carvalhosa, Ana B. Aouba, Achille Damaj, Gandhi Canioni, Danielle Brouzes, Chantal Gyan, Emmanuel Durupt, Stéphane Durieu, Isabelle Cathebras, Pascal Costédoat-Chalumeau, Nathalie Launay, David Pilmis, Benoit Barete, Stephane Frenzel, Laurent Lortholary, Olivier Hermine, Olivier Hermans, Cedric Chandesris, Marie-Olivia A French National Survey on Clotting Disorders in Mastocytosis |
title | A French National Survey on Clotting Disorders in Mastocytosis |
title_full | A French National Survey on Clotting Disorders in Mastocytosis |
title_fullStr | A French National Survey on Clotting Disorders in Mastocytosis |
title_full_unstemmed | A French National Survey on Clotting Disorders in Mastocytosis |
title_short | A French National Survey on Clotting Disorders in Mastocytosis |
title_sort | french national survey on clotting disorders in mastocytosis |
topic | 4800 |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4616764/ https://www.ncbi.nlm.nih.gov/pubmed/26447996 http://dx.doi.org/10.1097/MD.0000000000001414 |
work_keys_str_mv | AT carvalhosaanab afrenchnationalsurveyonclottingdisordersinmastocytosis AT aoubaachille afrenchnationalsurveyonclottingdisordersinmastocytosis AT damajgandhi afrenchnationalsurveyonclottingdisordersinmastocytosis AT canionidanielle afrenchnationalsurveyonclottingdisordersinmastocytosis AT brouzeschantal afrenchnationalsurveyonclottingdisordersinmastocytosis AT gyanemmanuel afrenchnationalsurveyonclottingdisordersinmastocytosis AT duruptstephane afrenchnationalsurveyonclottingdisordersinmastocytosis AT durieuisabelle afrenchnationalsurveyonclottingdisordersinmastocytosis AT cathebraspascal afrenchnationalsurveyonclottingdisordersinmastocytosis AT costedoatchalumeaunathalie afrenchnationalsurveyonclottingdisordersinmastocytosis AT launaydavid afrenchnationalsurveyonclottingdisordersinmastocytosis AT pilmisbenoit afrenchnationalsurveyonclottingdisordersinmastocytosis AT baretestephane afrenchnationalsurveyonclottingdisordersinmastocytosis AT frenzellaurent afrenchnationalsurveyonclottingdisordersinmastocytosis AT lortholaryolivier afrenchnationalsurveyonclottingdisordersinmastocytosis AT hermineolivier afrenchnationalsurveyonclottingdisordersinmastocytosis AT hermanscedric afrenchnationalsurveyonclottingdisordersinmastocytosis AT chandesrismarieolivia afrenchnationalsurveyonclottingdisordersinmastocytosis AT carvalhosaanab frenchnationalsurveyonclottingdisordersinmastocytosis AT aoubaachille frenchnationalsurveyonclottingdisordersinmastocytosis AT damajgandhi frenchnationalsurveyonclottingdisordersinmastocytosis AT canionidanielle frenchnationalsurveyonclottingdisordersinmastocytosis AT brouzeschantal frenchnationalsurveyonclottingdisordersinmastocytosis AT gyanemmanuel frenchnationalsurveyonclottingdisordersinmastocytosis AT duruptstephane frenchnationalsurveyonclottingdisordersinmastocytosis AT durieuisabelle frenchnationalsurveyonclottingdisordersinmastocytosis AT cathebraspascal frenchnationalsurveyonclottingdisordersinmastocytosis AT costedoatchalumeaunathalie frenchnationalsurveyonclottingdisordersinmastocytosis AT launaydavid frenchnationalsurveyonclottingdisordersinmastocytosis AT pilmisbenoit frenchnationalsurveyonclottingdisordersinmastocytosis AT baretestephane frenchnationalsurveyonclottingdisordersinmastocytosis AT frenzellaurent frenchnationalsurveyonclottingdisordersinmastocytosis AT lortholaryolivier frenchnationalsurveyonclottingdisordersinmastocytosis AT hermineolivier frenchnationalsurveyonclottingdisordersinmastocytosis AT hermanscedric frenchnationalsurveyonclottingdisordersinmastocytosis AT chandesrismarieolivia frenchnationalsurveyonclottingdisordersinmastocytosis |