Immune

Thrombocytopenia

Presented by:
Dr Mehroze Fatima
 Immune thrombocytopenia

• It is an autoimmune condition in which pathogenic antibodies bind to

platelets accelerating their clearance from the circulation.
 Etiology
• Primary (idiopathic)ITP:
It is mediated by autoantibodies, most often directed against the platelet membrane
glycoprotein IIb/IIIA, which sensitize the platelets resulting in premature removal
from the circulation by cells of the reticuloendothelial system.
-Disorder is primary idiopathic in most adults

• Secondary ITP:
It can be associated with autoimmune disease such as SLE, lymphoproliferative
diseases such as B cell lymphoma, infections caused by HEP C virus, HIV and
H.Pylori.
-here in addition to antiplatelet antibodies additional mechanisms may be
involved(such as direct suppression of platelet production by HIV, and cirrhosis
related decreased TPO production and secondry splenomegaly due to HEP c virus)
• Drug related ITP: Results from drug-platelet interactions prompting
antibody binding. Medications includes:
o Quinine and quinidine,
o Platelet inhibitors (abciximab, tirofiban)
o Antibiotics (linezolid, rifampin, sulfonamides and vancomycin)
o Anticonvulsants: phenytoin, valporic acid and carbamezapine)
o Analgesics (acetaminophen, naproxen and diclofenac)
o Cimetidine,chlorothiazide
 Clinical features

• Presentation depends upon the degree of thrombocytopenia.

• Spontaneous bleeding typically occurs only when platelet count has
fallen below 10,000-20,000mcL(10-20 x 10 9/L).
• At higher counts, patient may complain of easy bruising, gingival
bleeding ,spontaneous epistaxis or menorrhagia.
• Individuals with secondary ITP(such as due to autoimmune disease,
HIV or HCV infection, SLE, or lymphoproliferative malignancy) may
have additional disease specific findings.
Investigations and Diagnosis
• Primary ITP is the diagnosis of exclusion
• Laboratory test do not confirm the diagnosis of primary ITP, although they
help to exclude the secondary causes.
• Primary ITP has scenario of isolated thrombocytopenia in the absence of
underlying causative disease or medication
• CBC (thrombocytopenia)
• Peripheral blood smear: confirm for automated platelet count, asses for platelet
clumping, and to determine platelet ,red cell, and white cell morphologies.
• Tests for infection associated causes(HIV,HCV,HBV)
• Bone marrow in patients with unexplained cytopenias in two or more lineages,
cases not responding to immune suppression therapy, or atypical signs and
symptoms
• Serologic tests for antiplatelet antibodies generally do not help in diagnosis
 Management
• Decision to treat primary ITP depends on severity of thrombocytopenia and bleeding risk
• Individuals with platelet count less than 25,000-30,000/mcl or those with significant
bleeding should be treated.
• Remainder may be monitored serially for progression(Blood counts are recommended
every 3-6 months, and the patient should be instructed to consult in case of bleeding,
surgery or invasive procedure and pregnancy)

1- Initial therapy consists of Prednisolone 1 mg/kg/day orally for 10-14 days followed
by taper
OR
Dexamethasone 40mg/day or orally for 4 days.(every 2-4weeks
for 4-6 cycles) with or without
IVIG 1 g/kg/day intravenously for 2 days or
ANTI-D(WinRho)75mcg/kg intravenously for 1 dose(use in non
splenectomised,Rh +ve blood type, non anemic patients only)
Platelet transfusions may be given in case of bleeding.
• Over two third of patients will respond to initial therapy with oral corticosteroids
while most relapse following taper.
• Patients with persistent platelet count of less than 30,000/mcl or clinically
significant bleeding are candidates for 2nd line
therapy.
2- Second line therapies:
• Rituximab 375mg/m2 Iv weekly for 4 weeks OR
• Thrombopoieten receptor agonists:
• Romiplostim (1 mcg/kg subcutaneously weekly)
• Eltrombopag ( 25mg orally daily)
• Avatrombopag ( 20 mg orally daily initially)

• Fostamitinib (spleen tyrosine kinase inhibitor) 100mg orally twice

daily for patients who do not respond to corticosteroids, TPO agonists
or Rituximab.
3- Splenectomy:

• Management of secondry ITP may include a combination of treatment for the

underlying disease and therapies similar to those for primary ITP.
Splenectomy

• It has a response rate of over 50% and may be considered

for cases of severe ITP that fail to respond to initial
treatment or are refractory to second line agents.
• If available laparoscopic Splenectomy is preferred.
• Patients should receive pneumococcal ,Haemophilus
influenza type b and Meningococcal vaccination at least 2
weeks before therapeutic Splenectomy.
Splenectomy vaccination protocol
• Get at least one shot of all the vaccines before surgery and then get the
second shot or booster after surgery.
• PCV-13 (2 doses required) - 1st dose 2 weeks before surgery and second
one after 8 weeks from the 1st dose. (PPSV23 is not available in Pakistan).
• Meningococcal ACWY (Menctra) 2 dose series. 1st dose 1 week after the
PCV-13 and 2 weeks before surgery and then second dose at 8 weeks from
the first dose.
• Hexaxim (DTaP + HiB + Hep B + iPV) 3 dose series. 1 dose with PCV13
and then surgery after atleast 2 weeks from the first dose, second dose at 6
weeks from the first dose and then 3rd dose @ 12-14 weeks from the first
dose.
• Yearly Influenza vaccine in Sep to Oct.
• Complete 2 dose series of COVID-19 vaccine as well.
Thank you