Pediatr. praxi. 2022;23(5):344-347

A sleepy newborn with severe hemophilia A

MUDr. Veronika Fiamoli, Ph.D.

Oddělení dětské hematologie a biochemie, FN Brno

Hemophilia A is a rare X-linked hereditary bleeding disorder, characterized by a deficiency in factor VIII clotting activity. Up to 3.5 to 4% of newborn boys with hemophilia may develop intracranial bleeding. Intensive treatment of this bleeding with a factor VIII concetrates may lead to the inhibitor development. The treatment of choice in patients with a factor VIII inhibitor is the bispecific monoclonal antibody emicizumab. We present a case report of a boy with severe hemophilia A with delayed intracranial hemorrhage requiring extensive neurosurgery and intensive factor VIII replacement therapy that led to the inhibitor development. Despite the serious clinical condition, the treatment was successful and the boy is now without any alteration of psychomotor development.

Keywords: hemophilia, intracranial bleeding, factor VIII inhibitor, emicizumab.

Accepted: October 4, 2022; Published: October 11, 2022  Show citation