Name – Sumit Kumar Abhinav
Group - 1527
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Idiopathic (Immune) Thrombocytopenic Purpura презентация
Содержание
- 2. Idiopathic (Immune)
- 3. Statistics of ITP Incidence of 22 million/year
- 5. Clinical Manifestations May be acute or insidious
- 8. Etiology of ITP : Children Often after
- 9. Diagnosis (of Exclusion) Rule out other causes:
- 10. Diagnosis (of Exclusion) Rule out other causes:
- 11. To Marrow or Not to Marrow? Bone
- 12. Anti-Platelet Antibody Testing NOT recommended by ASH
- 13. Management of ITP Goal = prevention of bleeding, NOT cure!
- 14. General Principles of Therapy Major bleeding rare
- 15. “Safe” Platelet Counts “moderately” t-penic = 30-50,000
- 16. When Planning Therapy… Tailor therapy and decision
- 17. Initial Therapy Prednisone 1 mg/kg/day
- 18. Second-Line Therapy IV Immune Globulin (IVIg)
- 19. Treatment Side-Effects Steroids *bone density
- 20. Splenectomy Usually reserved for treatment failure
- 21. Data from George, JN, Woolf, SH, Raskob,
- 22. Response Post-Splenectomy Usually normalized PLTs within 2
- 23. Data from Fabris, F, et al. Br J Haematol 2001; 112:637.
- 24. Chronic Refractory ITP Persistent > 3 months PLT < 50,000 Failure to respond to splenectomy
- 25. When all else fails… Steroids IVIg /
- 26. Wrapping it up… ITP is often a
- 28. Return to Main
Слайд 2Idiopathic (Immune)
Thrombocytopenic Purpura
Thrombocytopenia in
the absence of other blood cell abnormalities (normal RBC & WBC, normal peripheral smear)
No clinically apparent conditions or medications that can account for thrombocytopenia
No clinically apparent conditions or medications that can account for thrombocytopenia
Слайд 3Statistics of ITP
Incidence of 22 million/year in one study
Prevalence greater as
often chronic
*Segal et al ?100 million/year
*age-adjusted prevalence 9.5/100,000
*1.9 :1 females / males
*Segal et al ?100 million/year
*age-adjusted prevalence 9.5/100,000
*1.9 :1 females / males
Слайд 5Clinical Manifestations
May be acute or insidious onset
Mucocutaneous Bleeding
*petechiae, purpura,
ecchymosis
*epistaxis, gum bleeding
*menorrhagia
*GI bleed, CNS bleed = RARE
*epistaxis, gum bleeding
*menorrhagia
*GI bleed, CNS bleed = RARE
Слайд 8Etiology of ITP : Children
Often after infection (viral or bacterial)
Theories:
*antibody cross-reactivity
*H. pylori
*bacterial lipopolysaccharides
*H. pylori
*bacterial lipopolysaccharides
Слайд 9Diagnosis (of Exclusion)
Rule out other causes:
*lab error / PLT
clumping
*drug / medication interaction
*infections (HIV, Hepatitis C)
*thyroid / autoimmune disease
*destructive / consumptive processes (TTP/HUS)
*bone marrow disease (leukemias, MDS)
*drug / medication interaction
*infections (HIV, Hepatitis C)
*thyroid / autoimmune disease
*destructive / consumptive processes (TTP/HUS)
*bone marrow disease (leukemias, MDS)
Слайд 10Diagnosis (of Exclusion)
Rule out other causes:
*lab error / PLT
clumping
*drug / medication interaction
*infections (HIV, Hepatitis C)
*thyroid / autoimmune disease
*destructive / consumptive processes (TTP/HUS)
*bone marrow disease (leukemias, MDS)
*drug / medication interaction
*infections (HIV, Hepatitis C)
*thyroid / autoimmune disease
*destructive / consumptive processes (TTP/HUS)
*bone marrow disease (leukemias, MDS)
Слайд 11To Marrow or Not to Marrow?
Bone marrow aspiration & biopsy if…
Patient
60 yrs. or older
Poorly responsive to tx
Unclear clinical picture
Poorly responsive to tx
Unclear clinical picture
Слайд 12Anti-Platelet Antibody Testing
NOT recommended by ASH Practice Guidelines
Poor positive/negative predictive values,
poor sensitivity with all current testing methods…
…and doesn’t change the management!
…and doesn’t change the management!
Слайд 14General Principles of Therapy
Major bleeding rare if PLT > 10,000
Goal =
get PLT count to safe level to prevent bleeding…
…not to specifically cure the ITP!
…not to specifically cure the ITP!
Слайд 15“Safe” Platelet Counts
“moderately” t-penic = 30-50,000
Probably safe if asymptomatic
Caution with elderly
(CNS bleeds)
Слайд 16When Planning Therapy…
Tailor therapy and decision to treat to the individual
patient
Weigh bleeding vs. therapy risks
Weigh bleeding vs. therapy risks
Слайд 17Initial Therapy
Prednisone 1 mg/kg/day
*usually response within 2 weeks
Taper off
after PLT response
Duration of use = controversial
Duration of use = controversial
Слайд 18Second-Line Therapy
IV Immune Globulin (IVIg)
1 gram/kg/day x 2 days
WinRho
(anti-D) – if pt is Rh+
50-75 mcg/kg/day
50-75 mcg/kg/day
Слайд 19Treatment Side-Effects
Steroids
*bone density loss *GI
effects
*muscle weakness *weight gain
IVIG/anti-D
*hypersensitivity *headache
*renal failure *nausea/vomiting
*alloimmune hemolysis
*muscle weakness *weight gain
IVIG/anti-D
*hypersensitivity *headache
*renal failure *nausea/vomiting
*alloimmune hemolysis
Слайд 20Splenectomy
Usually reserved for treatment failure
Consider risk of bleeding, pt lifestyle
RISKS
*surgical procedure
*loss of immune function ? vaccinations
*loss of immune function ? vaccinations
Слайд 21Data from George, JN, Woolf, SH, Raskob, GE, et al. Blood
1996; 88:3.
When to do Splenectomy?
Слайд 22Response Post-Splenectomy
Usually normalized PLTs within 2 weeks (often immediately)
Younger pts do
better
Kojouri et al (Blood 2004) ? 65% CR
Kojouri et al (Blood 2004) ? 65% CR
Слайд 25When all else fails…
Steroids
IVIg / anti-D
Rituximab (anti-CD20)
Cyclophosphamide
Danazol
Accessory splenectomy
H. pylori eradication
Слайд 26Wrapping it up…
ITP is often a chronic disease in adults
Multiple therapies
may be needed over time
Goal = prevention of complications
Therapy needs to be tailored to the individual patient
Goal = prevention of complications
Therapy needs to be tailored to the individual patient
