Idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura (ITP)- Adalah kondisi jumlah platel rendah penyebab nya idiophatic.- Berhubungan dengan antibodies terhadap platel.- ITP is also known as immune thrombocytopenic purpura or immune-mediated

thrombocytopenic purpura.Children ITP

- Onset nya akut dan pulih sendiri dalam 6 bulan

Adult ITPEtiologi:Autoimun terhadap trombhositPhatophysiologi:

Signs and symptomsSymptom:

Reaksi abnormal sel Thelper dgn antigen thrombosit pd permukaan apc

Auto Ab IgG pada membrane glikoprotein platelet IIb-IIa

Opsonisasi & fagositosis trombhosit makrofag spleen dan hati

thrombositopenia

Merusak megakaryocte

Kompensasi BM:>>produksi giant platel -> GIANT PLATEL

Perdarahan mikrovaskulerBleeding time naik

Mukosa:

GUMBLEEEDING

EPISTAKSIS

KULIT: PETECHIAE

Epidemiology:

Sex In chronic ITP (adults), the female-to-male ratio is 2.6:1. More than 72% of patients

older than 10 years are female. In acute ITP (children), distribution is equal between males (52%) and females (48%).

Age Peak prevalence occurs in adults aged 20-50 years. Peak prevalence occurs in children aged 2-4 years.

Approximately 40% of all patients are younger than 10 years.

Tanda dan gejala:

purpura and petechiae :

- Jika platelet < 20.000 permm3 : terjadi purpura dan peteche especially on the extremities, bleeding from the nostrils, bleeding at the gums, and menorrhagia

- A very low count (<10,000 per mm3) may result in the formation of hematomas in the mouth or on other mucous membranes.

- Mechanism:Peningkatan tekanan pada jarinagan>> merusak kapiler di bawah kulit>>darah kluar dri PD>> trombositopeni>> timbul lah bruise (purpura)

Epistaksis:

- Kerusakan endothel PD pleksus dan a. ethmoidalis anterior>>trombhositopeni>>darah keluar secara diapedesis melalui membrane basalis>>perdarahan delayed n prolong>>epistaksis

Fibrinogen meningkat:

- Seharusnya fibrinogen berikatan dengan permukaan trombosit.

Bleeding time meningkat.

PEMERIKSAAN FISIK * Non palpable petechiae, which mostly occur in dependent regions * PERDARAHAN BULLAE ( CAIRAN SEROSA) on mucous membranes * Purpura * Gingival bleeding * Signs of GI bleeding * Menometrorrhagia, menorrhagia * Retinal hemorrhages * Evidence of intracranial hemorrhage, with possible neurologic symptoms * Nonpalpable spleen: The prevalence of palpable spleen in patients with ITP is approximately the same as that in the non-ITP population (ie, 3% in adults, 12% in children). * Spontaneous bleeding when platelet count is less than 20,000/mm3.

GAMBAR : PERDARAHAN BULLAE

DDDisseminated Intravascular CoagulationHIV Infection and AIDSThrombocytopenic Purpura

PEMERIKSAAN LABCBC :

- Adanya trombositopenia pada pemeriksaan lab- Giant platel- menghitung WBC dan hemoglobin biasanya normal, kecuali perdarahan parah telah terjadi.

SX: PEM. SUTUL, ANTIBODI ANTIPLATEL, ANTIBODI IgG

MANAGEMENT Pasien yang asymptomatic,mild or moderate ITP no treatment Pasien no bleeding:

o Plt > 50 = tidak di terapio Plt 20-50 = monitoro Plt <20 = prednisone OR IV Ig

Emergency treatment karena perdarahan akut yang disebabkan oleh trombositopeni berat:

o Platelet transfusion ( emergency)o IV Ig infusion di ikuti platelet transfusiono High dose glucocoticoid

Initial management of ITP adults w/symptomatic purpura:o Plt>10 prednison sajao Plt<10 prednison + IV Ig

Prinsip penatalaksanaa epistaksis:o ABCo Hentikan perdarahandengan di tekano Msh, berikan adrenalin 1:1000,oxymetazolin semprot hidung) ke daerah

perdarahn.

Complications

Complications of idiopathic thrombocytopenic purpura may include the following:

Intracranial or other major hemorrhage Severe blood loss

Adverse effects of corticosteroids

Pneumococcal infections if the patient must have a splenectomy

Prognosis

Childreno Approximately 83% of children have a spontaneous remission, and

89% of children eventually recover.

o More than 50% of patients recover within 4-8 weeks.

o Approximately 2% of patients die.

Adults

o Only 2% of adults have a spontaneous recovery; however, approximately 64% of adults eventually recover.

o Approximately 30% of patients have chronic disease, and 5% of patients die from hemorrhage.

Patient Education

Instruct patients to return for follow-up in order to assess for a potentially reduced platelet count.

Emphasize close outpatient follow-up care.

Because of the increased risk of bleeding, instruct patients to avoid aspirin products.