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Pulmonary manifestation of a condition resembling Kasabach–Merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report

INTRODUCTION: Kasabach–Merritt syndrome is a benign condition characterized by hemangiomatosis, severely disseminated intravascular consumption coagulopathy, and thrombocytopenia. The mortality rate increases from 12% to 30% in hemorrhagic cases. In general, the symptoms primarily manifest in the ga...

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Autores principales: Cheufou, Danjouma Housmanou, Hager, Thomas, Welter, Stefan, Theegarten, Dirk, Stamatis, Georgios
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4436809/
https://www.ncbi.nlm.nih.gov/pubmed/25956942
http://dx.doi.org/10.1186/s13256-015-0566-z
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author Cheufou, Danjouma Housmanou
Hager, Thomas
Welter, Stefan
Theegarten, Dirk
Stamatis, Georgios
author_facet Cheufou, Danjouma Housmanou
Hager, Thomas
Welter, Stefan
Theegarten, Dirk
Stamatis, Georgios
author_sort Cheufou, Danjouma Housmanou
collection PubMed
description INTRODUCTION: Kasabach–Merritt syndrome is a benign condition characterized by hemangiomatosis, severely disseminated intravascular consumption coagulopathy, and thrombocytopenia. The mortality rate increases from 12% to 30% in hemorrhagic cases. In general, the symptoms primarily manifest in the gastrointestinal tract, the skin, and the subcutaneous tissue. There is no publication about pulmonary manifestation of angiomatosis in combination with vascular malformation and hemoptysis. This is the first description of a Kasabach–Merritt syndrome-like condition in the lung. CASE PRESENTATION: We present the case of a 29-year-old German woman with angiomatosis and associated pulmonary vascular malformation in her lower left lobe with a Kasabach–Merritt syndrome like condition. It was detected after hemoptysis. We also present our case observations and management. CONCLUSION: In a case of angiomatosis with an associated pulmonary lobar vascular malformation, lobectomy can be safely performed to prevent life-threatening bleeding.
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spelling pubmed-44368092015-05-20 Pulmonary manifestation of a condition resembling Kasabach–Merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report Cheufou, Danjouma Housmanou Hager, Thomas Welter, Stefan Theegarten, Dirk Stamatis, Georgios J Med Case Rep Case Report INTRODUCTION: Kasabach–Merritt syndrome is a benign condition characterized by hemangiomatosis, severely disseminated intravascular consumption coagulopathy, and thrombocytopenia. The mortality rate increases from 12% to 30% in hemorrhagic cases. In general, the symptoms primarily manifest in the gastrointestinal tract, the skin, and the subcutaneous tissue. There is no publication about pulmonary manifestation of angiomatosis in combination with vascular malformation and hemoptysis. This is the first description of a Kasabach–Merritt syndrome-like condition in the lung. CASE PRESENTATION: We present the case of a 29-year-old German woman with angiomatosis and associated pulmonary vascular malformation in her lower left lobe with a Kasabach–Merritt syndrome like condition. It was detected after hemoptysis. We also present our case observations and management. CONCLUSION: In a case of angiomatosis with an associated pulmonary lobar vascular malformation, lobectomy can be safely performed to prevent life-threatening bleeding. BioMed Central 2015-05-09 /pmc/articles/PMC4436809/ /pubmed/25956942 http://dx.doi.org/10.1186/s13256-015-0566-z Text en © Cheufou et al.; licensee BioMed Central. 2015 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Cheufou, Danjouma Housmanou
Hager, Thomas
Welter, Stefan
Theegarten, Dirk
Stamatis, Georgios
Pulmonary manifestation of a condition resembling Kasabach–Merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report
title Pulmonary manifestation of a condition resembling Kasabach–Merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report
title_full Pulmonary manifestation of a condition resembling Kasabach–Merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report
title_fullStr Pulmonary manifestation of a condition resembling Kasabach–Merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report
title_full_unstemmed Pulmonary manifestation of a condition resembling Kasabach–Merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report
title_short Pulmonary manifestation of a condition resembling Kasabach–Merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report
title_sort pulmonary manifestation of a condition resembling kasabach–merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4436809/
https://www.ncbi.nlm.nih.gov/pubmed/25956942
http://dx.doi.org/10.1186/s13256-015-0566-z
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