IDIOPATHIC

IDIOPATHIC (AUTOIMMUNE)
(AUTOIMMUNE)
THROMBOCYTOPENIC
THROMBOCYTOPENIC PURPURA
PURPURA
PRAYER AMID COVID-19 CRISIS

Heavenly Father, You know what situation we are in right now. But we believe that You are
always in control. We thank You oh Lord and we entrust everything to You.

We pray for our Leaders and for our Government. Give them wisdom as they make decisions
for the whole country.

We pray for all Health Workers who always put themselves on risk to save and cure the
patients. Protect them Lord from any sickness. Give them strength to endure daily hardships
and capacity to cure people.

We also pray for all the police officers and military and all the front liners. Protect them oh
Lord and give them strength and patience.
PRAYER AMID COVID-19 CRISIS

And lastly, we pray for the entire nation Lord. We pray for provision for our daily needs
especially for the poor. We pray for healing, protection and deliverance. We pray for all the
businesses to survive this crisis. We pray for recovery and restoration.

We rest in Your loving embrace because You are the God of mercy, of healing, and of
provision. This we pray in Jesus’ Name, Amen.
OBJECTIVES:

1. To define what Idiopathic Thrombocytopenic Purpura/ITP

is and its etiology and epidemiology.
2. To differentiate acute and chronic ITP and their clinical
manifestations.
3. To discuss simply its pathophysiology, diagnostics, and
management.
 IDIOPATHIC (AUTOIMMUNE)
THROMBOCYTOPENIC PURPURA
• Most common cause of acute thrombocytopenia in an otherwise well child
• Epidemiology: 1 in 20,000
• Etiology: unknown
recent viral infection, 1-4 wks (50-65%)
autoantibodies against platelet glycoprotein complexes – ɑIIb-ß3 and GPIb
Pathogenesis
Immune
Viral Infection Complexes on the
Platelet Surface

Destruction and
removal by the
Thrombocytopenia
reticuloendothelial
system
 *Ab-coated platelets are recognized by Fc receptor on the splenic macrophages, thus, are ingested and
destroyed.
CLINICAL MANIFESTATIONS:
 Sudden onset of generalized petechiae and purpura in a
previously healthy child
 Mucocutaneous bleeding
 Profound thrombocytopenia
(Plt <10 x 109/L)
* Splenomegaly, lymphadenopathy,
bone pain, pallor – rare
UNITED KINGDOM CLASSIFICATION SYSTEM

1. No symptoms
2. Mild (bruising and petechiae, occasional minor epistaxis)
3. Moderate (more severe skin and mucosal lesions,
menorrhagia)
4. Severe (menorrhagia, melena, epistaxis, symptoms interfere
with life)
 Prognosis
70-80% - spontaneous remissions w/in 6 mo
<1% - intracranial hemorrhage
10-20% - develop chronic ITP

Acute ITP (80%) – complete resolution of platelet CT in 6 mos.

Chronic ITP (20%) – persistence of thrombocytopenia > 12mos
– monitor for SLE ( ANA )
– 50% in adolescents
Lab. Findings:
thrombocytopenia <20 x 109/L
platelet size - normal to large platelets
normal WBC , RBC

BMA : increased megakaryocytes,

normal granulocytes, erythrocytes
Indications for bone marrow aspiration
1. Abnormal WBC count or differential count
2. Unexplained anemia
3. Findings suggestive of BM disease

Direct Antiglobulin Test / Coombs Test

= to rule out Evans Syndrome (autoimmune hemolytic anemia plus
thrombocytopenia)
In adolescents with new-onset ITP, ANA test should be done to
evaluate for SLE
• TREATMENT:

1. No therapy other than education and counseling of

family and patients for patients with mild, minimal
and moderate symptoms.
2. IVIG
• “A single dose of IVIG (0.8-1.0g/kg for 1-2 days) or a short
course of corticosteroids should be used as a first line
treatment.” -American Society of Hematology Guidelines
• IVIG at a dose of 0.8-1.0 g/kg/day for 1-2 days induces a rapid
rise in platelet count (usually >20 x 109/L) in 95% of patients
within 48 hr.
3. Prednisone.
• 1-4mg/kg/24hr
• Usually continued for short course until a rise in
platelet count to >20 x 109/L
4. Intravenous anti-D therapy.
• For Rh-positive patients, IV anti-D at a dose of 50-75
μg/kg causes a rise in platelet count to >20 x 109/L in
80-90% of patients within 48-72 hr.
• The role of Splenectomy in ITP should be reserved for
2 circumstances:
a. Older child (>4 y/o) with severe ITP that has lasted
more than one year and symptoms are not easily
controlled
b. Intracranial hemorrhage

• Splenectomy is successful in inducing complete

remission in 64-88% of children with chronic ITP.
• Rituximab – alternative for splenectomy which
induces a partial or complete remission to 30-40%
children.
• Romiplastin & Eltrombopag – two effective agents
that act to stimulate thrombopoiesis that are
approved by the FDA to treat adults with chronic ITP
 Management of ITP
• Treatment goal for ITP is to get safe platelet levels and prevent bleeding.
• American Society of Hematology gives the following guidelines for the
treatment of ITP;
– Don’t treat if,
• Platelet count > 50,000 and patient is asymptomatic.
– Consider treatment if;
• Platelet count > 50,000 and there is mucous membrane bleeding.
– Definitely treat if;
• Platelet count < 50,000
• Thank you!! 