Afibrinogenemia & Important Manifestations/Complications

• Afibrinogenemia is an inherited Quantitative Autosomal Recessive disorder 

• Plasma and platelet levels of the fibrinogen are not measurable 
• Plasma levels <0.1 g/L or <10 mg/dL

• Homozygous or Compound heterozygous

• FGA gene is most commonly involved

• Clinical manifestations

• Bleeding

• Neonatal bleedings like umbilical stump bleeding

• Gastrointestinal bleeding

• Urological bleeding 

• Central nervous sytsem bleeding

• Spleen (with splenic rupture)

• Abnormal wound healing

• Obstetric complications

• 1^st trimester fetal loss

• Antepartum hemorrhage

• Postpartum hemorrhage

• Spontaneous abortion

• Placental abruption

• Bone cysts

• Mostly in long bones → possible effects of bleeding

NOTE: Arterial or venous thrombosis is possible