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Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging

Kasabach-Merritt phenomenon (KMP) is characterized by vascular tumour and consumptive coagulopathy with life-threatening thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of high fibrin split products. We report a case of 3-year-old bo...

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Autores principales: Keskindemirci, Gonca, Tuğcu, Deniz, Aydoğan, Gönül, Akçay, Arzu, Aktay Ayaz, Nuray, Er, Ali, Yekeler, Ensar, Bilgiç, Bilge
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4578750/
https://www.ncbi.nlm.nih.gov/pubmed/26435870
http://dx.doi.org/10.1155/2015/537530
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author Keskindemirci, Gonca
Tuğcu, Deniz
Aydoğan, Gönül
Akçay, Arzu
Aktay Ayaz, Nuray
Er, Ali
Yekeler, Ensar
Bilgiç, Bilge
author_facet Keskindemirci, Gonca
Tuğcu, Deniz
Aydoğan, Gönül
Akçay, Arzu
Aktay Ayaz, Nuray
Er, Ali
Yekeler, Ensar
Bilgiç, Bilge
author_sort Keskindemirci, Gonca
collection PubMed
description Kasabach-Merritt phenomenon (KMP) is characterized by vascular tumour and consumptive coagulopathy with life-threatening thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of high fibrin split products. We report a case of 3-year-old boy with local aggressive vascular lesions associated with KMP. Magnetic resonance imaging revealed an extensive lesion at paravertebral and retroperitoneal regions that was infiltrating vertebrae. Although we did not get any response to steroid or propranolol treatment, partial response was observed radiologically with interferon-alpha treatment. Unfortunately, the patient died because of the uncontrolled consumptive coagulopathy that led to intracranial hemorrhage which was caused by huge knee hematoma after minor trauma.
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spelling pubmed-45787502015-10-04 Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging Keskindemirci, Gonca Tuğcu, Deniz Aydoğan, Gönül Akçay, Arzu Aktay Ayaz, Nuray Er, Ali Yekeler, Ensar Bilgiç, Bilge Case Rep Pediatr Case Report Kasabach-Merritt phenomenon (KMP) is characterized by vascular tumour and consumptive coagulopathy with life-threatening thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of high fibrin split products. We report a case of 3-year-old boy with local aggressive vascular lesions associated with KMP. Magnetic resonance imaging revealed an extensive lesion at paravertebral and retroperitoneal regions that was infiltrating vertebrae. Although we did not get any response to steroid or propranolol treatment, partial response was observed radiologically with interferon-alpha treatment. Unfortunately, the patient died because of the uncontrolled consumptive coagulopathy that led to intracranial hemorrhage which was caused by huge knee hematoma after minor trauma. Hindawi Publishing Corporation 2015 2015-09-08 /pmc/articles/PMC4578750/ /pubmed/26435870 http://dx.doi.org/10.1155/2015/537530 Text en Copyright © 2015 Gonca Keskindemirci et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Keskindemirci, Gonca
Tuğcu, Deniz
Aydoğan, Gönül
Akçay, Arzu
Aktay Ayaz, Nuray
Er, Ali
Yekeler, Ensar
Bilgiç, Bilge
Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging
title Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging
title_full Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging
title_fullStr Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging
title_full_unstemmed Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging
title_short Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging
title_sort paravertebral and retroperitoneal vascular tumour presenting with kasabach-merritt phenomenon in childhood, diagnosed with magnetic resonance imaging
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4578750/
https://www.ncbi.nlm.nih.gov/pubmed/26435870
http://dx.doi.org/10.1155/2015/537530
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