diathesis haemorrhagis

DIATHESIS

HAEMORRHAGIS

VASKULER TROMBOSIT

FAKTOR.PEMBEKUAN

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

1. Vaskuler : Resistensi dan kontraktilitasnya yang normal,

dengan susunan jaringan penunjang yang

adekuat

2. Trombosit : Jumlah yang cukup, kualitas dan aktifitas yang

normal

3. Faktor koagulasi : Kadar yang normal untuk membuat bekuan

darah menjadi stabil

Vaskuler.

Berperan : - melakukan vasokonstriksi

- sel endotel vaskuler memproduksi bahan vaso aktif yang

merangsang terjadinya aggregasi trombosit

Trombosit.

- Umur : 7 – 10 hari

- Fungsi :

- Adhesi : trombosit melekat pada endotel

- Agregasi : trombosis melekat sesamanya membentuk thrombus

- Pembebasan kolagen dan tromboxane yang - vasokonstriksi &

agregasi

- Fusi ireversibel

DIATHESIS HEMORRHAGISDIATHESIS HEMORRHAGIS

• KELAINAN :

1. VASKULER

2. TROMBOSIT

3. FAKTOR PEMBEKUAN

1. Kelainan pembuluh darah :

a. Herediter : - teleangiektasi herediter

b. Didapat : - purpura simpleks

- purpura senilis

- purpura anafilaktoid ( Henoch-Schonlein)

- scurvy

K elainan trombosit :

a. Trombositopeni : - hipoplasi sumsum tulang

- leukemia

- mieloma multipel

b. Tromboaestenia : gangguan aggregasi

trombosit (herediter)

Kelainan faktor pembekuan :

- Hemofilia (defisiensi F.P VIII)

- Parahemofilia (defisiensi F P IX)

- Penyakit Von Willebrand

- Defisiensi vitamin K. : Biasanya pada bayi baru lahir

5Kelainan Faktor Pembekuan 5Kelainan Faktor Pembekuan dengan Kel Vaskuler dan Trombdengan Kel Vaskuler dan Tromb

Kel F.Pemb Kel Vask& TromKel F.Pemb Kel Vask& Trom

1.Epid : Turunan Turunan/didapat1.Epid : Turunan Turunan/didapat2. Kelainan : Haematom Purpura/Ekimosis2. Kelainan : Haematom Purpura/Ekimosis3. Trauma : + - 3. Trauma : + - 4. Lab : W.pemb > W.perd >4. Lab : W.pemb > W.perd >

ITPITP

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.

PathophysiologyPathophysiology

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).

TREATMENTTREATMENT

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

.

ManagementManagementThe 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.

TreatmentTreatment

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