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KASABACH MERRITT SYNDROME: MANAGEMENT WITH INTERFERON
Kasabach Merritt Syndrome (KMS) is a rare, locally aggressive, vascular tumor. The objectives of treatment of KMS are to prevent bleeding from consumptive coagulopathy and induce vascular tumor regression. A 14-month old female child was brought with a reddish lesion on the left scapular area notice...
Autores principales: | , , , , |
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Formato: | Texto |
Lenguaje: | English |
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Medknow Publications
2010
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2965921/ https://www.ncbi.nlm.nih.gov/pubmed/21063527 http://dx.doi.org/10.4103/0019-5154.70705 |
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author | Acharya, Sandhya Pillai, Kalyani Francis, Abel Criton, S Parvathi, V K |
author_facet | Acharya, Sandhya Pillai, Kalyani Francis, Abel Criton, S Parvathi, V K |
author_sort | Acharya, Sandhya |
collection | PubMed |
description | Kasabach Merritt Syndrome (KMS) is a rare, locally aggressive, vascular tumor. The objectives of treatment of KMS are to prevent bleeding from consumptive coagulopathy and induce vascular tumor regression. A 14-month old female child was brought with a reddish lesion on the left scapular area noticed at birth, which suddenly increased in size since 3 days. Hemogram revealed anemia severe thrombocytopenia, prolongation of bleeding, clotting time and increased fibrin degradable products, suggestive of KMS. Coagulopathy was managed by transfusing fresh frozen plasma and platelets. Oral prednisolone up to 5mg/kg/day for four weeks yielded no effect on thrombocytopenia or regression of tumor size. Embolization of feeding artery was attempted but not feasible. We used Interferon –alpha– 2b (IFN α 2b), in a dosage of 3million IU/m(2) /day subcutaneously. Within a month the platelet count increased and the vascular tumor started regressing. This case signifies the importance of step wise management of KMS. |
format | Text |
id | pubmed-2965921 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2010 |
publisher | Medknow Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-29659212010-11-09 KASABACH MERRITT SYNDROME: MANAGEMENT WITH INTERFERON Acharya, Sandhya Pillai, Kalyani Francis, Abel Criton, S Parvathi, V K Indian J Dermatol Case Report Kasabach Merritt Syndrome (KMS) is a rare, locally aggressive, vascular tumor. The objectives of treatment of KMS are to prevent bleeding from consumptive coagulopathy and induce vascular tumor regression. A 14-month old female child was brought with a reddish lesion on the left scapular area noticed at birth, which suddenly increased in size since 3 days. Hemogram revealed anemia severe thrombocytopenia, prolongation of bleeding, clotting time and increased fibrin degradable products, suggestive of KMS. Coagulopathy was managed by transfusing fresh frozen plasma and platelets. Oral prednisolone up to 5mg/kg/day for four weeks yielded no effect on thrombocytopenia or regression of tumor size. Embolization of feeding artery was attempted but not feasible. We used Interferon –alpha– 2b (IFN α 2b), in a dosage of 3million IU/m(2) /day subcutaneously. Within a month the platelet count increased and the vascular tumor started regressing. This case signifies the importance of step wise management of KMS. Medknow Publications 2010 /pmc/articles/PMC2965921/ /pubmed/21063527 http://dx.doi.org/10.4103/0019-5154.70705 Text en © Indian Journal of Dermatology http://creativecommons.org/licenses/by/2.0/ This is an open-access article distributed under the terms of 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 Acharya, Sandhya Pillai, Kalyani Francis, Abel Criton, S Parvathi, V K KASABACH MERRITT SYNDROME: MANAGEMENT WITH INTERFERON |
title | KASABACH MERRITT SYNDROME: MANAGEMENT WITH INTERFERON |
title_full | KASABACH MERRITT SYNDROME: MANAGEMENT WITH INTERFERON |
title_fullStr | KASABACH MERRITT SYNDROME: MANAGEMENT WITH INTERFERON |
title_full_unstemmed | KASABACH MERRITT SYNDROME: MANAGEMENT WITH INTERFERON |
title_short | KASABACH MERRITT SYNDROME: MANAGEMENT WITH INTERFERON |
title_sort | kasabach merritt syndrome: management with interferon |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2965921/ https://www.ncbi.nlm.nih.gov/pubmed/21063527 http://dx.doi.org/10.4103/0019-5154.70705 |
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