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Case Rep Pediatr. 2015;2015:537530. doi: 10.1155/2015/537530. Epub 2015 Sep 08.

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

Case reports in pediatrics

Gonca Keskindemirci, Deniz Tuğcu, Gönül Aydoğan, Arzu Akçay, Nuray Aktay Ayaz, Ali Er, Ensar Yekeler, Bilge Bilgiç

Affiliations

  1. Department of Pediatric Hematology-Oncology, ?stanbul Kanuni Sultan Süleyman Educational and Research Hospital, 34303 Istanbul, Turkey.
  2. Department of Pediatric Hematology-Oncology, Faculty of Medicine, Ac?badem University, 34742 Istanbul, Turkey.
  3. Department of Pediatric Rheumatology, ?stanbul Kanuni Sultan Süleyman Educational and Research Hospital, 34303 Istanbul, Turkey.
  4. Department of Radiology, ?stanbul Kanuni Sultan Süleyman Educational and Research Hospital, 34303 Istanbul, Turkey.
  5. Department of Radiology, ?stanbul Faculty of Medicine, ?stanbul University, 34093 Istanbul, Turkey.
  6. Department of Pathology, ?stanbul Faculty of Medicine, ?stanbul University, 34093 Istanbul, Turkey.

PMID: 26435870 PMCID: PMC4578750 DOI: 10.1155/2015/537530

Abstract

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.

References

  1. Br J Haematol. 2001 Mar;112(4):851-62 - PubMed
  2. J Med Imaging Radiat Oncol. 2009 Feb;53(1):87-91 - PubMed
  3. Int J Clin Exp Med. 2013 Oct 25;6(10):851-60 - PubMed
  4. Eur J Pediatr Surg. 2006 Oct;16(5):369-72 - PubMed
  5. J Pediatr. 1996 Dec;129(6):947-8 - PubMed
  6. Pediatr Blood Cancer. 2007 Oct 15;49(5):739-44 - PubMed
  7. Pediatr Blood Cancer. 2015 Mar;62(3):536-8 - PubMed
  8. Acta Oncol. 1995;34(6):735-40 - PubMed
  9. Pediatr Hematol Oncol. 1993 Apr-Jun;10(2):197-8 - PubMed
  10. J Pediatr Surg. 1993 Oct;28(10):1356-7; discussion 1358-9 - PubMed
  11. J Spinal Cord Med. 2011;34(3):335-9 - PubMed
  12. BMC Pediatr. 2014 Jun 11;14:146 - PubMed
  13. Pediatrics. 2015 Jul;136(1):e203-14 - PubMed
  14. Head Neck. 2010 Aug;32(8):1088-98 - PubMed
  15. Ultrastruct Pathol. 2013 Dec;37(6):452-5 - PubMed
  16. Laryngoscope. 2004 Jul;114(7):1231-6 - PubMed
  17. Int J Dermatol. 2003 Sep;42(9):741-8 - PubMed

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