1. THROMBOCYTOPENIA Reduction in platelet count below 150 x 109
A smear of human perypheral blood, from : devobio/210labs/ct2.html

2. DIFFERENTIAL DIAGNOSIS OF THROMBOCYTOPENIA
Destructive Thrombocytopenia
Immunologic
ITP
Drug induced
Post-tranfusion purpura
Autoimmune disease
Post-transplant
Hyperthyroidism
Lymphoproliferative disorders

3. Nonimmunologic
Microanigopathic disease
Hemolytic anemia and thrombocytopenia
Hemolytic-uremic syndrome
Thrombotic thrombocytopenic purpura
Platelet Consumption/Destruction
DIC
Giant Hemangiomas
Cardiac (prosthetic heart valves, repair of intra
cardiac defects)

4. Neonatal Problems
Pulmonary hypertension
Polycythemia
RDS/infection (viral, bacterial, protozoal,
spirochetal)
Sepsis/DIC
Prematurity
Meconium aspiration
Glant hemangioma
Neonatal alloimmune
Neonatal autoimmune (maternal ITP)
Erythroblastosis fetalis (Rh incompatibility)

5. Impaired Production
Congenital & Hereditary Disorders
Thrombocytopenia-absent radii (TAR) syndrome
Fanconi’s anemia
Bernard-Soulier syndrome
Wiskott-Aldrich syndrome
Glanzmann’s thrombasthenia
May-Hegglin anomaly
Amegakaryocytosis (congenital)
Rubella syndrome
Associated with Chromosomal Defect
Trisomy 13 or 18

6. Metabolic disorders
Marrow infiltration malignancies, storage disease, myelofibrosis
Acquired processes
Aplastic anemia
Drug induced
Severe iron deficiency
Sequestration
Hypersplenism (portal hypertension, neoplastic, infectious, glycogen storage disease, cyanotic heart syndrome)
Hypothermia

7. Thrombocytopenia with neutropenia
Figure 1.
Approach to the child with
thrombocytopenia with neutropenia
or pancytopenia
History
Physical Examination
CBC : differential
platelet count
Peripheral smear evaluation
Thrombocytopenia with neutropenia
or pancytopenia
Consider :
Marrow infiltration
Marrow failure
hypersplenism
Asses:
Bone marrow aspirate
splenomegaly
Dd/:aplastik anemia
Fanconi’s anemia
Malignancy
Storage disease
Hypersplenisme

8. Approach to the child with isolated thrombocytopenia
Figure 2.
Approach to the child with
isolated thrombocytopenia
History
Physical Examination
CBC : differential
platelet count
Peripheral smear evaluation
Isolated thrombocytopenia
Asses : platelet morphology
normal
abnormal
Asses : splenomegaly or
lymphadenopathy
Bernard-Soulier syndrome
Wiskott-Aldrich syndrome
May-Hegglin anomaly
Glanzmann’s thrombasthenia
Gray platelet syndrome
positive or
negative
bone marrow aspirate
Normal examination
Congenital anomalies
dd/: malignancy
infection HIV
storage disease
hypersplenism
dd/: ITP (viral induced, drug induced)
HIV
Collagen vascular disease
Infant : alloimmune, autoimmune
Hemangiomas
Aplastic anemia
Fanconi’s anemia
Familial
skeletal abnormalitas
fanconi’s anemia
TAR
cyanotic heart disease
eczema : Wiskott-Aldrich syndrome
hemangiomas
Kasabach-Merritt syndrome

9. thrombocytopenia, anemia microangiopathic process
Figure 3.
Approach to the child with
thrombocytopenia and anemia
History
Physical Examination
CBC : differential
platelet count
Peripheral smear evaluation
thrombocytopenia, anemia
yes
Asses : intravascular hemolisis
red cell fragment
microangiopathic process
consumption no
Coombs’ test
dd/ : DIC
HUS
TTP
Sepsis
negative
positive
Reticulocyte count
dd/: Evans syndrome
HIV
EBV
Collagen vascular disease
Drug induced
high
normal or low
Assess physical
lihat figure 2
bone marrow aspirate
lihat figure 1

10. IMMUNE THROMBOCYTOPENIC PURPURA
= IDIOPATHIC THROMBOCYTOPENIA PURPURA
= AUTOIMMUNE THROMBOCYTOPENIC PURPURA
= MORBUS WERLHOF
= PURPURA HEMORRHAGICA

11. IDIOPATHIC THROMBOCYTOPENIC PURPURA ( ITP )
Acute ITP :
- resolve within 6 months
- usually occurs in children ages 2 to 4 years
Chronic ITP :
- persistent thrombocytopenia > 6 months
- child < 1 year or > 10 years of age
- may have an associated autoimmune disease or
immunodeficiency state
Neonatal Alloimmune Thrombocytopenic
Purpura ( NATP )

12. PATHOPHYSIOLOGY
Pathogenesis of epitop spread in ITP ( From Cines DB et al, Immune Thrombocytopenic Purpura, In : N Engl J med 2002 ; 346 )

13. CLINICAL MANIFESTATIONS
Sudden onset of generalized petechiae & purpura
Bleeding from the gums and mucous membrane
( paticularly with platelet < 10 x 109 / L )
History of preceding viral infection 1 to 4 week
before
No hepatosplenomegaly
1% of cases develop intracranial hemmorrhage
Resolution within 6 months

14. ptechiae purpura conjunctival hemorrhage Howell–Jolly bodies
( From Cines DB et al, Immune Thrombocytopenic Purpura, In : N Engl J med 2002 ; 346 )

15. LABORATORY FINDINGS Low platelet count Giant platelet
Hemoglobin maybe decreased if there have been
profuse bleeding
BMP : normal granulocytic & erythrocyte series
with characteristically normal or increased
numbers of megakaryocytes, some of the
megakaryocytes may appear to be immature
Platelet antibody (+)

16. TREATMENT Intravenous Immunoglobulin ( IVIG )
- Dose : 0,8 – 1 g / kg /day ; 1-2 days
- Expensive and time consuming to administer
- Adverse effect : headaches & vomitting suggestive of
aseptic meningitis
Prednison
- Dose : 1-4 mg / kg / 24 hours ; 2-3 week or until a rise in platelet count > / mm3
IV anti D therapy
- Given to Rh positive individuals
- Dose : 50 mikrogram / kg

17. Splenectomy
- The older children ( > 4 years ) with severe ITP
> 1 years ( chronic ITP )
- Life threatening hemorrhage ( intracranial
hemorrhage ), if the platelet count cannot
rapidly be corrected with tranfusion of platelets
and administration of IVIG and corticosteroids

18. Mechanisms of Action of Therapies for Immune Thrombocytopenic Purpura ( From Cines DB et al, Immune Thrombocytopenic Purpura, In : N Engl J med 2002 ; 346 )

19. NEONATAL ALLOIMMUNE THROMBOCYTOPENIA ( NATP )
Caused by the development of maternal antibodies
against antigens present on fetal platelets that are
shared with the father and recognized as foreign
by the maternal immune system
 platelet equivalent of Rh disease of the
newborn
1 in 4000 – 5000 live births

20. CLINICAL MANIFESTATIONS
Generalized petechiae & purpura within the first few
days after delivery
Normal maternal platelet count, moderate to severe
thrombocytopenia in the newborn
No maternal thrombocytopenia in the past
30 % severe NATP may develop intracranial
hemorrhage
Early jaundice occurs in 20 % of cases as a results of
resolution of intracranial or intraorgan hemorrhage
The thrombocytopenia is transient, lasting up to 3 to
6 weeks

21. DIFFERENTIAL DIAGNOSIS
Checking for the presence of maternal
alloantibodies directed against the father’s platelets
Specific studies to identify the target alloantigen
DIFFERENTIAL DIAGNOSIS
Transplacental transfer of maternal antiplatelet
auto antibodies ( Maternal ITP )
Viral or bacterial infection

22. TREATMENT
Transfusion with antigen – negatife patelets
IVIG ( 1 g / kg /24 hours, 1-2 doses until platelet
> / mm3 )
PREVENTION
IVIG 1 g / kg /week from midgestation until near
term
Delivery should be performed by cesarean
section

23. Terima Kasih