2. Refractory ITP patients should fulfill
2 criteria. First, they should have
failed splenectomy or have relapsed
thereafter. Second, they should either
exhibit severe ITP or have a risk of
bleeding that in the opinion of the
attending physician requires therapy

3. REFRACTORY ITP
 Two criteria have to be fulfilled at the same time: the
lack of response or relapse after splenectomy with
severe ITP or a bleeding risk that needs treatment
according to the GP
 Temporary response to corticosteroids or to
intravenous immunoglobulins does not exclude a
refractory form

4. TERMINOLOGY DISEASE DURATION
 Newly diagnosed (previously acute) < 3
months
 Persistent 3 to 12 months
 Chronic >12 months

5. Destruction of Platelets

6. CLINICAL FEATURES
 Easy or excessive bruising (purpura) — your skin naturally
bruises and bleeds more easily as you age, but this
shouldn't be confused with ITP
 Superficial bleeding into your skin that appears as a rash of
pinpoint-sized reddish-purple spots (petechiae), usually on
your lower legs
 Prolonged bleeding from cuts
 Spontaneous bleeding from nose
 Bleeding gums, especially after dental work
 Blood in urine or stools
 Unusually heavy menstrual flow
 Fatigue

9. Etiology of Thrombocytopenia
Decreased Production
 Dehydration, Vitamin B12 or folic acid deficiency
 Leukemia or myelodysplastic syndrome
 Decreased production of thrombopoietin by the liver in liver
failure
 Sepsis, systemic viral or bacterial infection
 Dengue fever can cause thrombocytopenia by direct infection of
bone marrow megakaryocytes, as well as immunological
shortened platelet survival.

10. Increased destruction of Platelets
 Thrombotic thrombocytopenic purpura
 Hemolytic-uremic syndrome
 Disseminated intravascular coagulation
 Paroxysmal nocturnal hemoglobinuria
 Antiphospholipid syndrome
 Systemic lupus erythematosus
 Post-transfusion purpura
 Neonatal alloimmune thrombocytopenia
 Splenic sequestration of platelets due to hypersplenism
 Dengue fever has been shown to cause shortened platelet survival and
immunological platelet destruction.
 HIV-associated thrombocytopenia[5]
 Gaucher's disease

11. TREATMENT

13. Thrombopoietin Receptor Agonist
Romiplostim:is a thrombopoiesis stimulating Fc-peptide
fusion protein (peptibody) that is administered by
subcutaneous injection.
ELTROMBOPAG:is an orally-administered agent with an
effect similar to that of romiplostim. It too has been
demonstrated to increase platelet counts and decrease
bleeding in a dose-dependent manner

17. Side effects of Splenectomy
Splenectomy increases the risk of sepsis due to
encapsulated organisms( S.Pneumoniae,
Haemophilus Influenzae).
Thus patient should be vaccinated with
Pneumococcal,Hib and Meningococcal vaccine.

18. Agent Dose
Acute (IV)
Methylprednisolone 30 mg/kg; max 1 g
IVIG 1 g/kg
Anti-D 75 μg/kg
Vincristine 0.03 mg/kg; max 1.5 mg
Vinblastine
10 mg (∼ 0.2 mg/kg)
Maintenance (po)
Danazol
10 mg/kg; round to the nearest 200
mg; 400-800 mg/day in adults
Azathioprine
2-2.5 mg/ kg; round to the nearest 50
mg; 100-200 mg/day in adults
Refractory ITP COMBINED Drugs

19. Reference
www.bloodjournal.org
www.mayoclinic.org

20. THANK YOU