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 ITP
Etiologi:
Autoimun terhadap trombhosit

Phatophysiologi:

Reaksi abnormal sel Thelper dgn antigen

thrombosit pd permukaan apc

Auto Ab IgG pada membrane

Merusak megakaryocte
glikoprotein platelet IIb-IIa

Opsonisasi & fagositosis trombhosit Kompensasi BM:>>produksi

makrofag spleen dan hati giant platel -> GIANT PLATEL

thrombositopenia

Bleeding time naik

Perdarahan mikrovaskuler

Mukosa:
KULIT: PETECHIAE
GUMBLEEEDING
Signs and symptoms
EPISTAKSIS
Symptom:
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

DD
Disseminated Intravascular Coagulation
HIV Infection and AIDS
Thrombocytopenic Purpura

PEMERIKSAAN LAB
CBC :
- 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 terapi
o Plt 20-50 = monitor
o 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 transfusion
o High dose glucocoticoid
 Initial management of ITP adults w/symptomatic purpura:
o Plt>10 prednison saja
o Plt<10 prednison + IV Ig
 Prinsip penatalaksanaa epistaksis:
o ABC
o Hentikan perdarahandengan di tekan
o 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
 Children
o 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.