| 6–11 [5] (3 patients) | Sickle cell trait* Haemophilia A | Bleed events, predominantly haemarthrosis, muscle haematomas, epistaxis, gum bleeds, gastrointestinal haemorrhage and haematuria Bleeding pattern similar to patients with haemophilia A only, but mean annual bleeding episodes significantly lower in those with sickle cell trait (31 +/- 5 vs 45 +/- 7; p=0.033) |
| 30 [6] | Sickle cell anaemia Haemophilia A (FVIII 28%) | Excessive bleeding following minor trauma in two maternal uncles Profuse bleeding after tooth extraction led to SCD diagnosis and internal haemorrhage after road traffic accident (1977) Two traumatic haematomas and a dental extraction treated with cryoprecipitate (1977–1986) Haematoma after blunt trauma treated with desmopressin (1986) Red cell transfusions for priapism (1989 and 1990) |
| 15 [7] | Sickle cell anaemia Haemophilia B (FVIII 51 IU/ ml, Factor IX 12%) Hepatitis B +ve & C +ve Prophylaxis with recombinant Factor IX since age 9; 3–5 infusions/ year for bleeds | Ten admissions before age two and five admissions in last 13 years for vasculo-occlusive crises and febrile illness Recurrent haemarthroses and mucosal bleeds due to active lifestyle |
| 19 [8] | Sickle cell disease/beta- thalassaemia Haemophilia A (FVIII <1%) | Father had beta-thalassemia trait, mother had sickle cell trait, sister was a beta-thalassemia carrier. No history of bleeding disorders SCD diagnosed when admitted with abdominal pain Presented five months later after bleeding for six days post-dental extraction; treated with tranexamic acid and fresh frozen plasma |
| 1.0, 2.5 [9] | Sickle cell disease Haemophilia A (FVIII <1%) (treatment not described) | No family history of sickle cell anaemia, but both disorders inherited from the mother Presented with post-circumcision bleeding No history of bleeding events |
