Showing posts with label Hematology. Show all posts
Showing posts with label Hematology. Show all posts

Monday, May 15, 2017

- Henoch-Schönlein Purpura (Anaphylctoid purpura)

Henoch-Schönlein Purpura (Anaphylctoid purpura)
Immune complex mediated disease contain IgA within capillaries of skin, GI and renal involvement.
Incidence:
  • HSP is the commonest generalized vasculitis of childhood.
  • HSP is the commonest cause of non-thrombocytopenic purpura in children.
  • Can occur at any age but peaking at 3–10 years of age.
  • Male : female = 2:1
  • Peaks during the winter months.
Etiology: the cause is unknown.
- It is postulated that genetic predisposition and antigen exposure increase circulating IgA levels and disrupt IgG synthesis.
-The IgA and IgG interact to produce complexes that activate complement and are deposited in affected organs, precipitating an inflammatory response with vasculitis.
- Antigen may be:
  • Infections (e.g. Group A ß hemolytic streptococci or ohers)
  •  Insect bites
  • Drugs or food allergy.
Pathology and pathogenesis:
  • IgA-mediated vasculitis of small blood vessels with IgA deposits seen in these vessels, primarily those of the skin and intestine.
  • Deposition of polymeric immunoglobulin A (IgA) in glomeruli. 
  • IgA deposits are present by immunofluorescence,
  •  Broad spectrum of glomerular lesions that can range from mild proliferation to necrotic and crescentic changes can be seen