a case report followed by some theory

1. IMMUNE
THROMBOCYTOPENIC
PURPURA (ITP)
By : FIKRI ABDULLAH ZAWAWI
KATHIRAVAN KANIASAN

2. CASE REPORT
Name : Kudryova Julie
Address : Kurovice 37,76s 52 Miskovice U
Holesova
Date of Birth : 31.5.2012
Weight : 18.5kg
Height : 105cm

3. Current Complaints
 She came to the hospital due to
planning of trepanobiopsy (bone
marrow aspiration and biopsy)
 Previously she was diagnosed with
chronic ITP
 She has weak cold and afebrile

4. Personal History
 Labor was spontaneous in 42nd week
 She has postpartum complication due
to knotted umbilical cord around her
neck
 She was in neonate ICU for 14 days
with ventilatory support
 She was breastfed for 8 months and
vaccinated according to schedule
 She had varicella infection at 8
months

5.  From September 2013 onwards her
mother noticed some bruising on her
body.
 In March 2014, she fell down and there
was a significant hematoma on her head
 She was diagnosed with ITP in July 2014
and acute bronchitis on October 2014
 She received IVIG against ITP
 In 26th January 2015 she was included in
the study of Romiplostim (protein
analogue of thrombopoietin)
 Last application was on 16th February
2016

6.  Allergy : No
 Pharmacologic History : No
 Social History: Sometimes she goes to
kindergarten otherwise at home.
Live with her family and has pet
Father is a smoker

7. Family History
 Her grandmother had valvular heart
disease
 Her mother has varicose vein
 Her father healthy
 Her older sister has migraine

8. Stomatic Status
 She was afebrile, with runny nose
 Normal heart sound, skin without
pathology, normal peristalsis, no
edema, no bruise
 She has slight splenomegaly

9. Laboratory Finding
 Leucocytes: 9.47 10^9/l
 Erythrocytes: 4.91 10^12/l
 Hemoglobin: 111g/L
 Hematocrit: 0.33
 MCV: 68.0 fL
 MCH: 22.6 pg
 RDW: 15.8%
 Platelets: 275 10^9

10. Differential Analysis
 Lymphocytes: 35.6%
 Monocytes: 9.3%
 Neutrophils: 52.6%
 Eosinophils: 2.3%
 Basophils: 0.2%

11. Theory Part

12. Immune Thrombocytopenic
Purpura(ITP)
 The most common cause of acute
onset of thrombocytopenia in an
otherwise well child
 Estimated about 1 in 20,000 children
 A recent history of viral illness is
described in 50-65% of cases of
childhood ITP

13.  One - 4 wk after exposure to a
common viral infection (eg EBV, CMV,
rhinitis, parvovirus B19)
 The peak age is 1-4 yr.
 ITP seems to occur more often in late
winter and spring after the peak
season of viral respiratory illness.

14. Pathophysiology
2 possible mechanisms :
1. An autoantibody bind to the platelet
surface, circulating antibody-coated platelets
are recognized by the Fc receptor on splenic
macrophages, ingested, and destroyed.
2. Impaired production of the glycoprotein
hormone thrombopoietin, which is the
stimulant for platelet production. (reduction in
circulating platelets)

15.  Source = https://undertheguiseofglitter.wordpress.com/2015/07/14/all-my-issues/

16. https://quizlet.com/95852614/pathophysiology-chapter-03-
hematopoietic-function-flash-cards/

17. Clinical Manifestation
 The classic presentation of ITP is a
previously healthy 1-4 yr old child who has
sudden onset of generalized petechiae and
purpura
 Often there is bleeding from the gums and
mucous membranes, particularly with
profound thrombocytopenia (platelet count
<10 × 10^9/L).
 The presence of abnormal findings such as
hepatosplenomegaly, bone or joint pain, or
remarkable lymphadenopathy suggests
other diagnoses

18. Classification System
 ITP is classified as:
Class 1: No symptomes
Class 2. Mild symptoms:
Bruising and petechiae
Occasional minor epistaxis
Very little interference with daily living
Class 3. Moderate:
More severe skin and mucosal lesions
More troublesome epistaxis and menorrhagia
Class 4. Severe:
Bleeding episodes—menorrhagia, epistaxis, melena—
requiring transfusion or hospitalization
- Symptoms interfering seriously with
the quality of life

20. Prognosis
 Severe bleeding is rare (<3% of
cases)
 In 70-80% of children who present
with acute ITP, spontaneous resolution
occurs within 6 mo
 Fewer than 1% of patients develop an
intracranial hemorrhage.
 Approximately 20% of children who
present with acute ITP go on to have
chronic ITP

21.  The outcome/prognosis may be
related more to age, as:
 ITP in younger children is more likely
to resolve
 The development of chronic ITP in
adolescents approaches 50%.

22. Differential Diagnosis
 Autoimmune thrombocytopenia may be an
initial manifestation of :
(Because ITP is a diagnosis of exclusion, it is
important to rule out other causes of low
platelets)
1. SLE
2. Infections (HIV, EBV, CMV, varicella, parvovirus
B19)
3. Common variable immunodeficiency
4. Hodgkin Lymphoma(rarely)
5. Vaccinations (eg, MMR )
6. Medications (anti-epilepsy)

23. 7. Congenital Amegakaryocytic
Thrombocytopenia (CAMT)
8. Bernard-Soulier Syndrome = (deficiency of
glycoprotein Ib (GpIb), the receptor for von Willebrand factor)
9. Wiskott-Aldrich Syndrome (WAS)=
(characterized by eczema, thrombocytopenia (low platelet
count), immune deficiency)
10. Glanzmann's thrombasthenia = (platelets
contain defective or low levels of glycoprotein IIb/IIIa (GpIIb/IIIa)
which is a receptor for fibrinogen. As a result, no fibrinogen
bridging of platelets to other platelets can occur, and the
bleeding time is significantly prolonged)

24. Management
 Initial approaches to the management
of ITP include the following:
1.No therapy other than education and
counseling of the family and patient for
patients with minimal, mild, and moderate
symptoms, as defined earlier.
2.Intravenous immunoglobulin (IVIG).
3.Intravenous anti-D therapy (for Rh
positive patients)

25. 4. Prednisone (continued for 2-3 wk or until a rise in
platelet count to >20 × 10^9/L achieved)
5. TPO mimetics (Romiplostim)
6. Mycophenolate mofetil (MMF)= for those
unresponsive to corticosteroids and/or splenectomy
7. Rituximab (monoclonal antibody against protein
CD20)
8. Immunosuppressant (azathioprine, cyclosporin
A)
9. Platelet transfusion in ITP is usually
contraindicated unless life-threatening
bleeding is present
10. Splenectomy in severe condition(age
above 6 y/old and other treatments are not effective –
before surgery need to give vaccination for
pneumococcal, meningococcal and h.influenza B)

26. Reference
 (Immune Thrombocytopenic Purpura (ITP): A New
Look at an Old Disorder, 2010), Published by
Indiana Hemophilia and Thrombosis Center
 http://www.itpsupport.org.uk/childhooditp.htm
 www.pdsa.org/about-itp.html
 http://www.bloodjournal.org/content/106/7/2244?ss
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