title: Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging.
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.
authors.affiliation: Department of Pediatric Hematology-Oncology, İstanbul Kanuni Sultan Süleyman Educational and Research Hospital, 34303 Istanbul, Turkey.
authors.forename: Gonca
authors.initials: G
authors.lastname: Keskindemirci
authors.affiliation: Department of Pediatric Hematology-Oncology, İstanbul Kanuni Sultan Süleyman Educational and Research Hospital, 34303 Istanbul, Turkey.
authors.forename: Deniz
authors.initials: D
authors.lastname: Tuğcu
authors.affiliation: Department of Pediatric Hematology-Oncology, İstanbul Kanuni Sultan Süleyman Educational and Research Hospital, 34303 Istanbul, Turkey.
authors.forename: Gönül
authors.initials: G
authors.lastname: Aydoğan
authors.affiliation: Department of Pediatric Hematology-Oncology, Faculty of Medicine, Acıbadem University, 34742 Istanbul, Turkey.
authors.forename: Arzu
authors.initials: A
authors.lastname: Akçay
authors.affiliation: Department of Pediatric Rheumatology, İstanbul Kanuni Sultan Süleyman Educational and Research Hospital, 34303 Istanbul, Turkey.
authors.forename: Nuray
authors.initials: N
authors.lastname: Aktay Ayaz
authors.affiliation: Department of Radiology, İstanbul Kanuni Sultan Süleyman Educational and Research Hospital, 34303 Istanbul, Turkey.
authors.forename: Ali
authors.initials: A
authors.lastname: Er
authors.affiliation: Department of Radiology, İstanbul Faculty of Medicine, İstanbul University, 34093 Istanbul, Turkey.
authors.forename: Ensar
authors.initials: E
authors.lastname: Yekeler
authors.affiliation: Department of Pathology, İstanbul Faculty of Medicine, İstanbul University, 34093 Istanbul, Turkey.
authors.forename: Bilge
authors.initials: B
authors.lastname: Bilgiç