A seminar on ITP

1. IDIOPATHIC
THROMBOCYTOPENIC
PURPURA (ITP)
SEMINAR
Presented by :Dr Shaz Pamangadan
Chair : Dr Balakrishnan Valliyot

2. ITP - IMMUNE/IDIOPATHIC
THROMBOCYTOPENIC PURPURA
• Definition: isolated thrombocytopenia with no clinically apparent
associated conditions or other causes of thrombocytopenia.
• Etiology: autoantibodies directed against platelets coat platelet
surface. IgG-coated platelets are taken up by RE system.
• Incidence: approximately 100 per million; half of these are
children. In adults, two peaks:
• one are young (<40) with female predominance,
• one are older (>60), no gender predominance.

4. CLASSIFICATION
1.Acute 2.Chronic
1.Primary ITP 2.Secondary ITP

11. PLATELET RESPONSE
•Platelets provide phospholipid
scaffold for thrombin generation.
•Platelets adhere
to vessel wall,
then aggregate,
leading to
formation of a
platelet plug

12. PATHOPHYSIOLOGY OF ITP

14. NORMAL PHYSIOLOGY-
PRODUCTION AND NUMBER
• Platelets are normally made in the bone
marrow from progenitor cells known as
megakaryocytes.
• Normal platelet lifespan is 10d. Every day,
1/10 of platelet pool is replenished.
• Normal platelet count is between 150,000 and
450,000/mm3

21. Differential Diagnosis
1)Pseudothrombocytopenia
2)Congenital thrombocytopenia (dohle bodies like inclusion in p.smear)
3)Acquired thrombocytopenia (Alcoholism)
4)TTP (schistocytes , raised LDH)
5)Malignant hypertension
6)DIC
7)Sepsis
8)Gestational thrombocytopenia
9)MDS
10)Type 2 B Von Willebrand disease
11)Splenomegaly/Hypersplenism
12)DIT
13)Severe Preeclampsia
14)HELLP Syndrome
15)A/C Fatty Liver of Pregnancy

22. EVALUATION OF PATIENT WITH
LOW PLATELETS
• History
• Has the patient ever had a normal platelet count?
• Carefully review medications.
• Antibiotics, quinine, anti-seizure medications
• Ask about other conditions which may be associated
with low platelets
• Liver Disease/hepatitis
• Thyroid Disease - both hypo- and hyper-
• Infections: viral, rickettsial
• Pregnancy
• Ask about other conditions which may be associated
with ITP
• Lupus, CLL, lymphoma

23. • Physical
• Evaluate for lymphadenopathy and splenomegaly
• Look for stigmata of bleeding
• Blood blisters and oral petechiae, ie “Wet Purpura”
• best harbinger of intracranial hemorrhage
• Laboratory Data
• Complete blood count
• Check B12 and folate levels.
• Send coagulation screens (PT/PTT) to exclude DIC
• Send HIV, hepatitis serologies and TSH
• Consider doing a bone marrow biopsy
• Megakaryocytes should be present.

24. P.smear  Isolated thrombocytopenia with normal RBC morphology
and larger-than-usual platelets but without any immature WBC series
fits the classic description of ITP.
ESR,ANA-collagen vascular disease
Platelet bound IgG antibody

25. PSEUDOTHROMBOCYTOPENIA
• Platelet clumping is of no clinical significance
• No increased risk of bleeding or clotting

26. Treatment Approach
Emergency treatment
IF ACTIVE BLEEDING PRESENT
combined therapeutic approach with platelet transfusion, a
corticosteroid (e.g., prednisone, methylprednisolone, or
dexamethasone), and intravenous immune globulin (IVIG).
Although platelets are likely to be rapidly destroyed during transfusion,
there is evidence to suggest that patients with active bleeding respond
transiently to transfusion.
IVIG can prolong platelet survival; therefore, platelet transfusion may be
more effective if given after IVIG infusion.
 Antifibrinolytic agents aminocaproic acid and tranexamic acid.

27. MANAGEMENT OF ITP
ASYMPTOMATIC ADULT
• If platelet count is >40-50 K, no therapy is
required. Check platelet counts at designated
intervals.
• If platelet count is < 20-30 K, begin therapy with
corticosteroids.
• Stop all NSAIDS and ASA to improve platelet
function.

28. INITIAL MANAGEMENT OF ITP
ADULT WITH SYMPTOMATIC PURPURA
• If platelet count is >10, treat with prednisone alone
- use 1 mg/kg.
• If platelet count <10, treat with prednisone, but
also add IVIg 1g/kg/d x 2d. - may require
admission
• Along with prednisone, add Calcium and Vitamin D
to prevent bone loss.
• If patient has severe bleeding, may need platelet
transfusions.

29. SUBSEQUENT MANAGEMENT OF ITP
ADULT WITH SYMPTOMATIC
PURPURA
• Follow platelet counts daily until >20, then can d/d
patient with close follow-up
• Once platelet count normalizes, commence a slow
steroid taper over 6-8 weeks.
• 1/3 patients  go into remission.
• 2/3 patients  relapse during or after steroid
tapering.

30. Management of Relapsed ITP
• Once the patient relapses, we may need to use
steroids to increase the platelet count out of the
danger range, but THIS CANNOT SUBSTITUTE
FOR DEFINITIVE THERAPY.
• Prednisone is now a crutch to support a dangerously
low platelet count.
• Options now include splenectomy (standard of care)
or intermittent treatment with anti-D immune
globulin (WinRho®).

31. MANAGEMENT OF RELAPSED ITP
SPLENECTOMY
• Splenectomy is effective in 2/3 of
patients, leading to normal platelet
counts.
• Can be performed via open method or
laparoscopically.
• Need to vaccinate against encapsulated
bacteria 2 weeks before procedure.
• May need steroids and/or IVIg before
procedure to boost platelet counts
preoperatively.

32. MANAGEMENT OF REFRACTORY ITP
• One third of patients will have an
inadequate response to splenectomy.
• Management of these patients involves
accepting that they have a chronic,
incurable condition.
• Target platelet counts should be lower--
aim for about 30K or absence of bleeding.

33. TREATMENT OF REFRACTORY ITP
• Immunosuppressive agents
• Rituximab (anti-CD20)
• No RTCs vs splenectomy
• 40% effective
• May be used before splenectomy
• Mycophenolate mofetil
• Cyclophosphamide
• Adjunct agents
• Thrombopoietin Receptor Agonists
• Romiplostim
• Eltrombopag

34. LATEST UPDATE (MARCH 2018)
Fostamatinib resulted in clinically meaningful, ongoing platelet
responses and a low rate of bleeding events in 43% of patients with
chronic immune thrombocytopenic purpura (ITP) with long-disease
duration who had been previously randomized to placebo in phase III
studies, according to the open-label extension trial Study