ITP parthenogenesis, causes, investigations and treatment modalities

1. Prepared by,
Anish Dhakal (Aryan)

2. Abnormal bleeding
Purpuric disorders
(primary hemostasis)
Disorders of
Coagulation (secondary
hemostasis)
Vessel problem Platelet problem
Abnormal function Decreased number
Congenital Acquired
Decreased production Increased destruction Sequestration
ITP

3. Normal Platelet count : (1.5 -4.5) *105/cu mm
Thrombocytopenia : Reduced platelets < 1.5 * 105/ cu mm
Causes of Thrombocytopenia :
•Decreased Production ( Congenital / Acquired)
•Sequestration of Platelets within enlarged spleen
•Increases destruction of normally synthesized platelets
1. Non- Immune
2. Immune
i. Idiopathic thrombocytopenic Purpura

4. Acute (<6 months)
Chronic (>6 months)
Commonest bleeding disorder
Presenting in children between 1-7 yrs age
Etiology
Cause is unknown
Common viruses in association with ITP:
Epstein Barr virus: usually of short duration
follows Infectious mononucleosis
Human Immuno Deficiency Virus: Chronic
Introduction

5. • Due to overactive immune system
Exposure to common viral infection
After 1-4 weeks
Autoantibody against antigen: Platelet Glycoprotein IIb/ IIIa
complex
Antibody coated Platelets circulate in blood
Recognized by Fc receptor on Splenic macrophages
Ingested
Destroyed
Pathogenesis

6. • Depends on platelets count.
• Mild unless platelets drop below 20,000 /cu mm
• Petechiae & Ecchymoses following mild trauma :20,000-
50,000 /cu mm
• Antecedent history of febrile illness.
• On presentation afebrile & well.
• Apperance of bruises & mucosal bleeding (Epistaxis/Oral
oozing)
• Prolonged bleed on superficial trauma.
Clinical manifestation

7. • No symptoms
• Mild symptoms :
• Bruising & petechiae
• Occasional minor epistaxis
• Very little interference with daily living
• Moderate:
• More severe skin and mucosal lesions
• More troublesome epistaxis
• Menorrhagia
• Severe: Bleeding episodes-
• Menorrhagia
• Epistaxis
• Melena
Classification of severity of bleeding:

8. • Normal with Petechiae & Purpura.
• Splenomegaly, lymphadenopathy or pallor rare.
•Fanconi anaemia
i. Hypoplasic/ Absent thumb
ii. Short stature
iii. Hyperpigmentation
•Hemangiomas: By USG of abdomen
•Collagen vascular disorder or malignancy
i. Splenomegaly & Lymphadenopathy
•Hypersplenism
Physical exam

9. 1. Complete Blood Count: Acute ITP
• Hb, WBC & differential count=Normal
• Hb may be decreased
2. Peripheral Blood Smear:
• Abnormal blast cells
• Malarial parasites
• To know precise platelets count
3. LFT & RFT and blood level of lactic dehydrogenase
• R/O hepatitis, hemolysis, HUS, Occult malignancy
Investigations

10. • Bone marrow Aspiration: Normal granulocytes and
erythrocytic series
Indications:
• Abnormal WBC count
• Unexplained anaemia
• Findings suggestive of Bone failure syndrome/ Malignancy
Hallmark: Increase in megakaryocyte number in the bone
marrow.

11. Other tests
• Antinuclear antibody test: for SLE
• HIV test
• Platelet antibody test: Acute ITP
• Direct Antiglobulin test (Coombs) : R/O Evans syndrome
Autoimmune hemolytic anemia and thrombocytopenia= Evans
Syndrome

12. Management
Goals of therapy
◦ Minimizing the risk of hemorrhage
◦ Decreasing long term side effects of treatment
In minimal, mild, and moderate symptoms no therapy only
counselling and close observation
In bleeding child
◦ Intravenous immunoglobulin (IVIG)
◦ Intravenous anti-D therapy
◦ Corticosteroid therapy
◦ Splenectomy

13. IVIG
dose of 0.8-1.0 g/kg/day for 1-2 days induces a rapid rise in platelet count in 95% of
patients within 48 hr.
IVIG appears to induce a response by down regulating Fc-mediated phagocytosis of
antibody-coated platelets.
IVIG therapy is both expensive and time-consuming to administer.
after infusion, there is a high frequency of headaches and vomiting, suggestive of
IVIG-induced aseptic meningitis.

14. IV anti-D.
For Rh positive patients, IV anti-D at a dose of 50-75 mg/kg causes a rise in platelet
count in 80-90% of patients within 48-72 hr.
RBC-antibody complexes bind to macrophage Fc receptors and interfere with
platelet destruction, thereby causing a rise in platelet count
But may induce mild hemolytic anemia
IV anti-D is ineffective in Rh negative patients

15. Corticosteroids
Steroids are presumed to reduce the risk of symptoms in ITP patients by:
◦ Reducing antibody production
◦ Reducing reticuloendothelial system phagocytosis of antibody-coated platelets
◦ Improving vascular integrity
Prednisone — 1 to 2 mg/kg (maximum dose 60 mg) per day in 3 divided doses for 14
days followed by a week of tapering
Methylprednisolone — 30 mg/kg per day intravenously for 3 days

16. Splenectomy
In child (≥4 yr) with severe ITP for >1 yr (chronic ITP) with uncontrolled symptoms
In life-threatening intracranial hemorrhage, if platelet count cannot be corrected
rapidly with transfusion of platelets and administration of IVIG and corticosteroids
High risk of post splenectomy infection caused by encapsulated organisms and
pulmonary HTN in adulthood

17. Chronic ITP
About 20% of patients with acute ITP have persistent thrombocytopenia for >12 mo
and are said to have chronic ITP
Splenectomy is successful in inducing complete remission in 64-88% of children with
chronic ITP
Alternate day low dose of steroids
Various combinations of danazol, vincristine, cyclosporine, azathioprine, Anti CD20
monoclonal Ab
To stimulate thrombopoiesis, romiplastin and eltrombopag

18. References:
OP Ghai, GHAI ESSENTIAL PEDIATRICS, 8th edition
Nelson’s Pediatrics, 20th edition