A 4 year-old male was brought to the emergency room with severe bleeding from nose. He had a complicated medical history significant for delayed umbilical stump detachment, intracerebral hemorrhage at birth, recurrent infections, misshapen brittle bones with several fractures in the past and poor wound healing with no pus formation. Blood analysis revealed leukocytosis and an autosomal recessive condition with mutations of FERMT3 gene, which encodes for Kindlin-3 protein. Bleeding stopped after the administration of red blood cell transfusions and recombinant Factor VIIa. What is the most likely diagnosis in this patient?
