DIATHESIS HAEMORRHAGIS

3555555555555555555555555555555555555 HEMOSTASE

TRIAS OF HEMOSTASE VASKULER TROMBOSIT FAKTOR.PEMBEKUAN

FAKTOR ---------- > HEMOSTASE

DIATHESIS HEMORRHAGIS KELAINAN : VASKULER TROMBOSIT FAKTOR PEMBEKUAN

KELAINAN HEMOSTASE

5Kelainan Faktor Pembekuan dengan Kel Vaskuler dan Tromb Kel F 5Kelainan Faktor Pembekuan dengan Kel Vaskuler dan Tromb Kel F.Pemb Kel Vask& Trom 1.Epid : Turunan Turunan/didapat 2. Kelainan : Haematom Purpura/Ekimosis 3. Trauma : + - 4. Lab : W.pemb > W.perd >

ITP 100 cases per 1 milion persons per year. Primary vs Secondary . Acute vs Chronic ( >6 months).

The etiology is still unknown and the pathogenesis is complex and possibly depends on disturbed antigen presentation, T cell activation and signaling, disregulated B cell stimulation and antibodies, unbalanced activation / suppression of complement.

In more than 70% of children, the illness resolves within six months, irrespective of whether they receive therapy. By contrast, ITP in adults is generally chronic.

The bone marrow in patients with ITP contains normal or increased numbers of megakaryocytes.

Pathophysiology ITP is mediated by IgG autoantibodies. Glycoprotein IIb/IIa, Ib/Ix, Ia/IIa, IV and V ... Accelerated clearance through Fcү receptors that are expressed by tissue macrophages (spleen & liver).

TREATMENT The decision to treat ITP is based on the platelet count, the degree of bleeding, and the patient’s lifestyle .

Management The incidence of intracranial hemorrhageis ~ between 0.2-1%. Almost all intracranial hemorrhages occur at platelet counts below 20.000/mm3, and generally below 10.000/mm3. Risk factors : head trauma and exposure to antiplatelet drugs.

Most children with typical acute ITP recover completely within a few weeks without treatment and that there is no proof that therapy prevents intracranial hemorrhage.

American Society of Hematology (ASH) recommends drug therapy for children with platelet counts of less than 10.000/mm3 with little or no purpura.

Treatment Watch & Wait strategy. Immunosupresive - Corticosteroids (high, standard or low dose). - Azathioprine IVIG (high or low dose, 2 day or 1 day). IV anti-D immunoglobulin in Rh(D) positive patients (high or low dose). Splenectomi