"ITP"

ITP
IDIOPATHIC
(AUTOIMMUNE)
THROMBOCYTOPENIC
PURPURA
DR. S. ASMA SHERAZI

ITP is the most common cause of acute onset of
thrombocytopenia in an otherwise well child.
EPIDEMIOLOGY
• Occurs 01 in 20,000 births.
• Usually 1-4wk after exposure to a common viral infection
(50% - 65% of cases).
• Male : Female equal in childhood.
• More often in late winter & spring (peak season of viral
respiratory illness).

PATHOGENESIS
• Idiopathic.
• Exact antigenic target for most such antibodies in most
cases of childhood acute ITP remains undetermined.
• In chronic ITP May demonstrate antibodies
against platelet glycoprotein.
complexes, αIIb-β, GPIb.

• Most common viruses which are associated with ITP are
EBV (short duration) & HIV (chronic)
Rarely in children infected with H. pylori and following
vaccines.

CLINICAL MANIFESTATIONS
• Classic presentation of ITP in previously healthy child is
sudden onset of petechiae and purpura.
• Parents often state that child was fine yesterday and
now covered with bruises and dots.
• There may be bleeding from the gums and mucous
membrane.
• Finding on physical examination are normal other than
petechiae and purpura.
• Splenomegaly, lymphadenopathy, bone pain, pallor are
rare.

CLASSIFICATION SYSTEM BY UNITED KINGDOM
To characterize the severity of bleeding in ITP.
NO
SYMPTOMS MILD MODERATE SEVERE
Bruising and
petechiae,
occasional minor
epistaxis, very
little interference
with daily living.
More severe skin
and mucosal
lesions, more
troublesome
epistaxis and
menorrhagia.
Bleeding
episodes –
Menorrhagia,
epistaxis, melena
– requiring
transfusion or
hospitalization,
symptoms
interfering
seriously with the
quality of life.

INVESTIGATIONS
CBC:
• Severe thrombocytopenia (platelet count < 20 ˣ109 /L).
• Platelet size normal or increase (reflective of increase
platelet turnover).
• Hemoglobin, WBC Count, Differential Count should be
normal. (Mild anemia may be due to bleeding).
PT & APTT:
• Normal.
BLEEDING TIME:
• Increased.

PERIPHERAL SMEAR:
• Thrombocytopenia, normal erythrocyte and leukocyte
count.
BONE MARROW ASPIRATION / BIOPSY:
• Normal granulocyte and erythrocte series, with normal or
increased numbers of megkaryocytes.
• Immature megakararyocyte may b present reflective of
increase platelet turnover.
HIV STUDIES:
• At risk population.

COOMBS TEST (Direct antiglobulin testing):
• If unexplained anemia to rule out Evans Syndrome
(autoimmune hemolytic anemia with thrombocytopenia).
OR
• Before giving therapy with IV anti-D.
PLATELET ANTIBODY TESTING:
• Seldom useful in acute ITP.
Other lab tests should be performed as indicated by history and
examination.

DIFFERENTIAL DIAGNOSIS
• Acquired Aplastic Anemia
• Myelodisplastic Syndrome
• Leukemia
• Lymphoma
• DIC
• Hemolytic Uremic Syndrome
• SLE, HIV infection
• Wiskott-Aldrich Syndrome (WAS)
CONGNITAL:
a. TAR (Thrombocytopenia-Absent Radius) Syndrome
b. MYH0-Related thrombocytopenia
• Exposure to radiations, medications and drugs
(Heparin induce thrombocytopenia)
• Insect bite

TREATMENT
• There are NO DATA showing that treatment affects either
short term or long term clinical outcome of ITP.
• Anti-platelet antibodies bind to transfused platelets as well
as autologus platelets.
SUPPORTIVE MEASURES:
• Reassurance.
• Restricting physical activity
and avioding trauma.
• Avoid medications that supress
platelet production or alter their
function e.g, aspirin, heparin,
valproic acid, digoxin etc.

PLATELET TRANSFUSION:
May be given in sever cases but destroy rapidly.
PHARMACOLOGICAL TREATMENT:
As per American Society of Hematology Guidelines:
“A single dose of IVIG (0.8-1g/kg) or short course of
corticosteroids should be use as first line treatment.”
IVIG at a dose of 0.8 – 1g/kg/day for 1-2 days induces a
rapid rise in platelets usually > 20 ˣ109 /L in 95% patient
within 48hours.
LIMITATION OF IVIG: Expensive, high frequency of
headaches and vomiting suggestive of IVIG-induced
aseptic meningitis.

PREDNISONE:
• Corticosteroids 1-4mg/kg/24hour
IV ANTI-D THERAPY:
• For Rh +ve patients.
• 50-75µg/kg causes rise in platelet count to > 20 ˣ109 /L
in 80-90% of patients within 48-72hours.
• Ineffective in Rh –ve patients.
Each of these medications may be used to treat ITP exacerbations,
which commonly occur several week after an initial course of therapy.

SPLENECTOMY:
Should be reserved for 1 0f 2 circumstances.
1) The older child (≥4yr) with severe ITP that
has lasted >1yr (chronic ITP).
2) Whose symptoms are not easily
controlled with therapy is a candidate
for spleenectomy.
Splenectomy must also be considered when
life-threatening hemorrhage (intracranial hemorrhage)
complicates acute ITP, if platelet count cannot be corrected
rapidly with transfusion of platelets and administration of
IVIG and corticosteroids

RISK AFTER SPLENECTOMY
Life long risk caused by encapsulated organisms,
increased risk of thrombosis, and potential development of
pulmonary hypertension in adulthood.
VACCINATION:
Administration of pneumococcal, meningococcal
and Hemophilus influenzae type b vaccines
prior to splenectomy recommended.
PROPHYYLAXIS:
Prophylactic penicillin following splenectomy should be
considered.

ALTERNATIVE TO SPLENECTOMY:
Rituximab used (off-label) in children to treat chronic ITP.
In 30-40% it has induced a partial or complete remission.
THROMBOPOIETIC AGENTS
Stimulate thrombopoiesis (in chronic ITP):
1) Romiplastin
2) Eltrombopag

REFERENCE:
Nelson Textbook of Pediatrics.

"ITP"

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