Hemophilia Royal Disease

Hemophilia

Also known as Hemophilia Royal Disease

word divide into two parts haem + philia = Hemophilia

1 Hem-blood

2 Philia-loving

Royal Disease

“Disorder which lovers to bleed” it is also called as Royal disease

is an x-chromosome-linked reserve disorder

In which there is excessive bleeding after an injury due to an absence of one or both clotting factor

In activity of platelets is normal but the activity of clotting factor and blood coagulation is disturb

Types

  • Mostly 5 types 
  1. hemophilia A
  • Cause
  • Clotting factor VIII absent
  • This is the most common
  1. Hemophilia B
  • Cause
  • Clotting factor IX absent
  • This  also was known as Christmas disease
  1. Hemophilia C
  • Cause
  • Clotting factor XI absent
  • This is the rarest type
  1. Para
  • Cause
  • Clotting factor V absent
  1. Acquired
  • Cause
  • Vitamin K deficiency {vit k (2,7,9,10)}
  • Autoimmune
  • Chronic liver failure
  • The rarest type

 

Clinical Features

 

◆ Prolonged bleeding after an injury

◆ Bleeding joint without any injury

◆ Ecchymosis joints

lab diagnosis

  • Bleeding time normal
  • Clotting time increase

Management

  •  Management for patient
  • Sports activity which can produce injury like football soccer cricket wrestling should be avoided
  • The patient is motivated for indoor activities like puzzles solving writing and video gaming
  • weight lifting exercises should be avoided and exercise which provides strength to the joints flexion and extension should be promoted
  • In acquired hemophilia, vitamin K rich diet and green leafy vegetables like spinach are advised to intake