An 8-year-old Girl with Intracranial Hemorrhage due to Acquired Factor XIII Deficiency as a Rare Complication of IgA Vasculitis: Case Report
Didem Avci Arvas1
, Enes Bıçaklıoğlu1, Gamze Nalbant1, Buse Berfin Çark1, Zeynep Tutar Çelik1, Cansu Durak2, Fitnat Uluğ3, Hüseyin Dağ1, Hasan Dursun4
1University Of Health Sciences, Prof. Dr. Cemiltaşcıoğlu City Hospital, Department Of Pediatrics, Istanbul, Türkiye
2University Of Health Sciences,sancaktepe Şehit Prof. Dr. İlhan Varank Training And Research Hospital, Pediatric Intensive Care Unit, Istanbul, Türkiye
3University Of Health Sciences, Prof. Dr. Cemiltaşcıoğlu City Hospital, Department Ofpediatric Neurology , Istanbul, Türkiye
4University Of Health Sciences, Prof. Dr. Cemiltaşcıoğlu City Hospital, Department Of Pediatric Nephrology, Istanbul, Türkiye
Keywords: Child, IgA vasculitis, Factor XIII deficiency, intracranial hemorrhage.
Abstract
IgA vasculitis (IgAV) is a complex immune-mediated vasculitis characterized by the involvement of small vessels, typically presenting in childhood. Patients often present with clinical findings, such as palpable purpura, gastrointestinal symptoms, renal involvement, and arthralgia. Rarely, central nervous system's vessels may also be involved, leading to intracranial hemorrhage. An 8-year-old girl admitted to the pediatrics department with a preliminary diagnosis of IgAV developed severe headaches, leading to cranial CT and MRI, which revealed intracranial hemorrhage (ICH). The patient was administered intravenous methylprednisolone at a dosage of 30 mg/kg/day for five days. Due to low Factor XIII and VIII levels, fresh frozen plasma was administered at 15 ml/kg. The patient showed clinical improvement and a significant reduction in ICH, leading to her discharge on the 21st day of hospitalization. According to our current knowledge in the literature, this case is the 6th case in children diagnosed with IgAV presenting with ICH, and the 2nd case presenting with ICH with low factor XIII levels. Mortality can be prevented if the rare complications of IgAV are recognised in time and with appropriate treatment modalities.
Cite this article as: Avci Arvas D, Bıçaklıoğlu E, Nalbant G, Çark BB, Tutar Çelik Z, Durak S, et al. An 8-year-old Girl with Intracranial Hemorrhage due to Acquired Factor XIII Deficiency as a Rare Complication of IgA Vasculitis: Case Report. Pediatr Acad Case Rep. 2025;4(2): 40-45.