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Von Wile brand disease

  1. Autosomal dominantQ
  2. Most common hereditary bleeding disorder Q
  3. Due to deficiency of Von Willebrand factor.

Clinical features

  1. Symptoms of mucocutaneous hemorrhage
  2. Since vWF is an acute phase protein stress will increase its level. Thus patient may not bleed with procedures that causes major stress, such as appendectomy and childbirth but may bleed excessively at the time of cosmetic or mucosal surgery. 

Lab finding

  1. Prolong bleeding time- platelets count normal
  2. Prolong PTT
    If these are thoughtful then vWF antigen vWF activity assay are to be done.


Immune thrombocytopenic purpura (ITP)
- is characterized by petechial hemorrhage, ecchymosis, thrombocytopenia reduced platelets survival, presence of platelets antibodies and a normal or increased number of megakaryocytes in the bone marrow. Anti platelets antibodies are commonly directed against the functional glycoprotein such as GPII/b IIIa.

Spleen is the major site of destruction when the IgG antibodies are involved.

 

Features

Acute

Chronic

 

Duration

4-6 wks

>6 months

 

Onset

Acute

Insidious

 

Peak age incidence

2-6year

All age

 

Sex predilection

None

F:M 3:1

 

Antecedent infection

Common

Unusual

 

Eosinophilia /Lymphocytosis

Common

Rare

 

Associated immunological abnormalities

None

20%

 

Spontaneous remission

80%

 Uncommon

 

 

Management-

  1. Intravenous immuno globin          
  2. Corticosteroid.
  3. Anti Rh (D) therapy                      
  4. Splenectomy              
  5. Supportive care




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