Normal Female Normal Male Victoria Edward Carrier Female Hemophiliac Male口 Victoria Albert 白 Alice Louis IV 210 Leopold Beatrice Helena 3 Alix Nicholas Il 4 Mary 78 自 Marie Alexis Tatiana Anastasia 1 Victoria 2 Edward vIl Juan Carlos 3 Irene Alexandra 10 4 George v all assassinated Charles 11 5 George VI Philip Elizabeth Il Diana 12 6 Margaret Andrew 13 7 Victoria Edward 14 8 Alfonso xill 13161415 Anne 15 Jua Sarah 16 William Harry
Hemophilia a and b Hemophilia a Hemophilia b Coagulation factor deficiency Factor VIII Factor IX Inheritance X-linked X-Iinked recessive recessive Incidence 1/10.000 males 1/100.000 males Severity Related to factor level <1%o-Severe -spontaneous bleeding 1-5%-Moderate-bleeding with mild injury 5-25%- Mild- bleeding with surgery or trauma Complications Soft tissue bleeding
Hemophilia A and B Hemophilia A Hemophilia B Coagulation factor deficiency Factor VIII Factor IX Inheritance X-linked X-linked recessive recessive Incidence 1/10,000 males 1/100,000 males Severity Related to factor level <1% - Severe - spontaneous bleeding 1-5% - Moderate - bleeding with mild injury 5-25% - Mild - bleeding with surgery or trauma Complications Soft tissue bleeding
FACTOR VI DEFICIENCY--HEMOPHILIAA Everity Factor VIII Clinical Features (or IX )level Severe <1% Spontaneous bleeding Moderate 1-5% Severe bleeding after trauma or surgery Mild 5-10% Moderate bleedit after trauma or surgery Subclinical 10-257 May not have symptoms
FACTOR VIII DEFICIENCY—HEMOPHILIA A Severity Factor VIII Clinical Features (or IX ) level Severe <1% Spontaneous bleeding Moderate 1-5% Severe bleeding after trauma or surgery Mild 5-10% Moderate bleeding after trauma or surgery Subclinical 10-25% May not have symptoms
Bleeds in Hemophilia Minor bleeds Oral mucosa Intra-articular Intramuscular Major bleeds Retroperitoneal Retropharyngeal Intracranial
Bleeds in Hemophilia • Minor Bleeds –Oral mucosa – Intra-articular – Intramuscular • Major Bleeds – Retroperitoneal – Retropharyngeal – Intracranial
Acutely bleeding in hemophilic can Compromise circulation Close off airway Lead to compartment syndrome ° Cause neuropath Cause pain Set up inflammatory synovitis, which can trigger more bleeding
Acutely, bleeding in hemophilics can • Compromise circulation. • Close off airway • Lead to compartment syndrome • Cause neuropathy • Cause pain • Set up inflammatory synovitis, which can trigger more bleeding