childhood ITP

1. Dr Kamrun Nahar
Pediatric hematology and oncology specialist

2. ITP
Most common cause of thrombocytopenia disorder in
children of any age.

3.  Epidemiology:
• 1 in 10,000 children per year
• Peak age incidence: 2-5 years of age
• Preceding viral prodromal is common
• 75% patient will present with platelets count >20,000/cu mm
• Majority spontaneously resolve.
spontaneous resolution occur in 60-80% of patients within 6-12 months from diagnosis

4. Late winter and early spring time

5. Aetiology:
• Unknown [ idiopathic]
• However, in about 50-60% cases platelet destruction may be follow
an episode of viral upper RTI about 2-3 weeks prior to bleeding
episodes.
• Viruses commonly involves are – HIV, EBV, Rubella, Varicella,
Measles, Parvo virus B19 and influenza.
• Bacteria commonly involves – H. pylori
Ahmolla 229

6. Pathogenesis of ITP:
• Heterogenous disease
• An acute infection is the initial
trigger but mostly it is a Auto
immune disorder
• Characterized by auto antibody
formation which interact with
glycoprotein on platelets and
megakaryocytes. Most common
glycoprotein includes GPIIb-IIIa,
GPIb-IX, GpIa-IIa,
• It results platelets destruction
followed by removed by spleen
and impaired thrombopoiesis.
• The net result is acute
thrombocytopenia

9. • Sudden onset of generalized skin bleeding in the form of petechiae,
purpura, echmysoses in a previously healthy child. Consequences and
sites of bleedings are like –
 Cutaneous – ‘ dry purpura’
– Petechiae, Purpura, Echymoses
– Easy bruishing
 Mucus -‘wet purpua’
– Nose (epistaxis)
– Eyes (conjunctiva)
– Mouth (from Buccal mucosa, gingival, palate, gum, tonsillar bed)
– Vagina (menorrhagia)
 Internal bleeding
– CNS (ICH) rare but fatal
– Abdomen (haematemesis), Rectum (malena or gross bleeding)
– Urinary tract (haematuria)
– Lungs (haemoptysis)
– Joints/muscle Bolton-Maggs, The Lancet, 1997

10. :
• Healthy non toxic child
• Have petechiae, purpura, ecchymosis
• May does not have pallor, it relates to
the degree of bleeding
• If pt present with
hepatosplenomegaly and/or
LAP it will be considered other
disease like Leukemia.
• If No congenital anomalies --
suggestive of IBFS

13. Differences between disorder of coagulation
& disorder of platelet or vessels
Types Disorders of
Coagulation
Disorders of platelet or
vessels
Onset of bleeding Insidious onset Acute onset
Petechiae Rare Characteristics
Mucosal bleeding Less common Common
Superficial ecchymosis Characteristics Rare
Hemarthrosis Characteristics Rare
Hematoma (deep
muscle)
Common Rare
Sex Hemophilia male
predominant
Chronic ITP common in
female
Positive family history Common Rare

14. Evaluation of ITP:
For diagnosis purpose:
1. CBC:
– Hb: usually normal unless haemorrhage is massive
– TC & DC of WBC: usually <50,000/cmm often more s <10,000/cmm
– Platelet: severely reduced (, even <20,000/c mm)
2. PBF:
• Platelet: May show larger platelets (mega platelet), otherwise normal.
• RBC normal & WBC normal, mature with no blasts.
3. MPV: normal or increased (reflecting normal or increased platelet turnover)
3. Platelet antibodies:
To exclude other disease:
• Coagulation profile: BT prolonged & PT, APTT are normal
• Bone marrow study: morphology or biopsy
For treatment purpose:
1. LFT, RFT
2. Blood grouping & Rh typing

15. Larger platelet size on peripheral smear
in ITP

16. Bone marrow study:
Findings in BM aspiration for morphology:
• Cellularity: normal
• Erythropoiesis: normal
• Granulopoiesis: normal
• Megakarytocyte: increased.
Indication of bone marrow in ITP:
• An abnormal WBC count or differential or unexplained anemia as
well as findings on history and physical examination suggestive of
Bone marrow failure syndrome or malignancy
• Cell lines other than platelets are affected
• Before staring steroid
• Severe pallor, hepatosplenomegaly, lymphadenopathy, bony
tenderness ---leukemia

17. Number of megakaryocytes slightly increased in an
otherwise normal bone marrow biopsy in ITP.

25. Bolton-magges et al. semin thromb and hemostasis,2001
To treat or not to treat:
Mild cases should not required rx.
Factors that influences decision to treat
• Parental anxiety
• Age of child
• Clinicians anxiety..

28.  Goal of therapy:
• Increase the platelet count enough to prevent serious hemorrhage,
possibly also to alleviate fatigue or difficulty of daily living.
 Treatment decision:
– Potential for bleeding
– Physical activity of level of patient
– Nature of bleeding
– Current platelet count
– Sign-symptoms
 Treatment options:
• No therapy with Close observation
• Drug treatment
ASH guidelines

29.  Counseling:
• Patient & parents about disease, its cause, progress, treatment
option, prognosis.
• Avoidance of Aspirin, NSAIDs
• Temporary restrictions from physical activities, contact sports
 What are the things to observe while patient is in without
treatment?:
1.Monitor new bleeding spots.
2.Platelet count
3.Vital signs
4.Any sign of other cell lineage disorder

34. Chronic ITP
 Defined as thrombocytopenia lasting for more than 12
months, who presented with acute ITP.
 Predictors of chronic ITP:
• Female gender
• Age >10 years at presentation
• Insidious onset of bruishing
• Presence of other auto antiboides eg SLE, HIV, and others
• Child may present with recurrent lowr RTI, GERD and FTT.
• Family history is often positive for ITP or other autoimmune
diseases (SLE, DM etc)

37. Others:
• Splenectomy & post-splenectomy penicilin prophylaxis.
• Monoclonal anti-B cell antibody e.g. Rituximab
• Drugs to stimulate thrombopoiesis e.g. Romiplostin, Eltrombopag
• Immunosuppresive therapy e.g. Azathioprine, Cyclosporin AH Molla 231

44. Acute ITP vs Chronic ITP
Traits Acute ITP Chronic ITP
Peak age of incidence Young children
(2-6 years)
Adolescence
Sex predilection None F:M = 2:1
Antecedent infection Common,
onset – acute insidious
Unusual
Haemorrhagic bullae
(in oral mucosa)
Common in severe
cases
Rare
Platelet count <20,000/mm3 30-80,000/mm3
Duration 2-8 wks >12 months
Spontaneous
remission
80% Uncommon

45. Take home message

46. Petechiae

47. Purpura

48. Ecchymosis

49. Approach to a patient with bleeding
PLATELET COUNT
BLEEDING TIME (BT)
PROTHROMBIN TIME (PT)
PARTIAL THROMBOPLASTIN TIME
(PTT)
THROMBIN TIME (TT)
SCREENING TESTS OF
BLEEDING
• Acute or chronic
• Wet or dry
• Medicine
• Look for other cell lineage disorder
• Family history
CBC & PBF

51. Thank you ..