title: Types of bleeding seen during the first 30 months of life in children with severe haemophilia A and B.
abstract: In view of excellent preservation of joint structure and function in Swedish patients started on primary prophylaxis at 1-2 years of age, it has recently been recommended that primary prophylaxis be considered optimal management for U.S. children with severe haemophilia (< 1% FVIII or FIX). In order to determine the age at which joint bleeding began, we reviewed (49) patients with severe haemophilia who were seen at our haemophilia centre for all bleeding episodes from birth on. Eight of the 49 children were first treated during the first few days of life for bleeding related to circumcision (6), spontaneous splenic rupture (1) or soft tissue haematoma (1). None had intracranial haemorrhage (ICH) in the immediate neonatal period. Other bleeding episodes for which children were treated during the first 30 months of life included soft tissue haematoma (42 episodes), tongue and mouth bleeding (37 episodes), ICH (four children) or for surgery (five children). Joint bleeding occurred in 16/49 children with severe haemophilia. Four of the 16 had haemophilia B, while 13 had haemophilia A. A single episode of severe joint bleeding occurred in each of the five patients before 12 months of age. Eight patients had 12 episodes of joint bleeding between 12 and 18 months of age and three patients accounted for 15 episodes between 18 and 24 months of age. Three children had a total of four joint haemorrhages between 25 and 30 months, and 33/49 children did not have any joint bleeding before 30 months of age. It is noteworthy that two children had recurrent bleeding into the same joint. One with haemophilia A had recurrent bleeding into the knees (at 14, 18, 20, 21 months), while one with haemophilia B had recurrent bleeding into one ankle starting at age 16 months. It would thus seem appropriate to begin primary prophylaxis in children with severe haemophilia once joint bleeding has begun (one or two episodes), rather than at a standard age.
authors.affiliation: Children's Hospital of Michigan, Detroit, Michigan, and Wayne State University School of Medicine, Detroit, Michigan, USA.
authors.forename: N
authors.initials: N
authors.lastname: Onwuzurike
authors.affiliation: Children's Hospital of Michigan, Detroit, Michigan, and Wayne State University School of Medicine, Detroit, Michigan, USA.
authors.forename: I
authors.initials: I
authors.lastname: Warrier
authors.affiliation: Children's Hospital of Michigan, Detroit, Michigan, and Wayne State University School of Medicine, Detroit, Michigan, USA.
authors.forename: J M
authors.initials: JM
authors.lastname: Lusher
keywords: acute haemarthrosis; bleeding manifestations; prophylaxis; soft tissue bleeding