Immune Thrombocytopenic Purpura

Max F J Mantik Sub Bag.Hematologi Onkologi Bag.IKA FK Unsrat/RSUP Manado

Synonim

Autoimmune thrombocytopenic purpura (AITP) adult chronic ITP Morbus werlhof Purpura hemorrhagica

ITP

Bleeding disorder
Acute, self limiting condition Recurrent

Chronic autoimmune disorder

Early destruction of platelet due to antibody binding removal platelet by the mononuclear phagocytic system

Incidence

ITP is more frequently diagnosed than any other form of destructive thrombocytopenia Acute form is more common in children, while chronic form affect young adults

ITP in children

Acute form
80 90% acute bleeding episode Resolving within a few days or weeks

Ending within 6 months

Both sexes equally affected Peek occurrence : 2 5 years of age History of viral, bacterial or vaccination Abrupt onset Platelet < 20 x 109/L

ITP in Children (2)

Chronic ITP
Insidious onset Predominance in female Most affected age > 7 years

Recurent form
Occur in 1 4% of children Episode of thrombocytopenia at interval > 3 months

Familial ITP
Rare disorder The nature is unclear

Pathogenesis

Increased rate of platelets destruction Bleeding occurs when platelet count fall < 10 50 x 109/L Evidence of platelet asociated IgG immune complexes opsonization destruction of platelets (Phagocytosis) Chronic ITP can be attributed to autoantibodies directed against platelet (glicoprotein)

Clinical features
Occurs within 1 3 weeks after an infectious diseases (bacterial or non specific viral infection) Common first bleeding sign :

bruising, petechia platelet count < 20 X 109/L Severe mucosal bleeding, hematuria, genital bleeding

platelet count < 10 x 109/L ICH occurs in 0.5 1 %

Apart from bleeding and thrombocytopenia no other abnormal physical findings Bone marrow examination is indicated to patients who fail to the treatment after 3 6 months

Clinical course

Complete remission occurs in 80 90% 3 5% of cases develop chronic ITP mostly over 7 years of age Recurrent ITP occurs in 14% children with acute ITP ICH occurs in 0.5 1% hospitalized children and 1/3 of those cases are fatal Post splenectomy sepsis is a cause of death in children with ITP

Management

Treatment is needed
Mucosal bleeding with platelet count < 20 x

109/L Minor bleeding with platelet count < 10 x 109/L

Main therapy : corticosteroid Other : IVIG, a-interferon, cytostatic agent, splenectomy.