Reduction in platelet count below 150 x 109

THROMBOCYTOPENIA
DIFFERENTIAL DIAGNOSIS OF THROMBOCYTO
PENIA
Destructive Thrombocytopenia
Immunologic
ITP
Drug induced
Post-tranfusion purpura
Autoimmune disease
Post-transplant
Hyperthyroidism
Lymphoproliferative disorders
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)
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)
Impaired Production
Congenital & Hereditary Disorders
Thrombocytopenia-absent radii (TAR) syndrome
Fanconis anemia
Bernard-Soulier syndrome
Wiskott-Aldrich syndrome
Glanzmanns thrombasthenia
May-Hegglin anomaly
Amegakaryocytosis (congenital)
Rubella syndrome
Associated with Chromosomal Defect
Trisomy 13 or 18
Metabolic disorders
Marrow infiltration mallgnancies, storage disease,
myelofibrosis
Acquirede processes
Aplastic anemia
Drug induced
Severe iron deficiency

Sequestration
Hypersplenism (portal hypertension, neoplastic,
infectious, glycogen storage disease, cyanotic
heart syndrome)
Hypothermia
IMMUNE THROMBOCYTOPENIC PURPURA

= IDIOPATHIC THROMBOCYTOPENIA PURPURA

= AUTOIMMUNE THROMBOCYTOPENIC PURPURA
= MORBUS WERLHOF
= PURPURA HEMORRHAGICA
 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 )
PATHOPHYSIOLOGY

Pathogenesis of epitop spread in ITP ( From Cines DB et al, Immune Thrombocytopenic

Purpura, In : N Engl J med 2002 ; 346 )
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
 Clinical Features of Immune Thrombocytopenic
Purpura.
Panel A shows extensive petechiae and purpura on the
legs of a child with immune thrombocytopenic
purpura. Whether children who present with only
these features should be treated is controversial. Panel
B shows a conjunctival hemorrhage. Extensive
mucocutaneous bleeding may be a harbinger of
internal bleeding. Typical changes after splenectomy
in the erythrocytes (arrow in Panel C) include pitting
and HowellJolly bodies (arrow in Panel D), which
are remnants of nuclear chromatin. Anterior view
(Panel E) and left lateral view (Panel F) of scans with
technetium Tc 99mlabeled heat-damaged red cells
show an accessory spleen (arrows) in a patient who
had a relapse of immune thrombocytopenic purpura
after splenectomy.

( From Cines DB et al, Immune Thrombocytopenic Purpura, In : N Engl J med 2002 ; 346 )
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 (+)
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 > 20.000 / mm3
IV anti D therapy

- Given to Rh positive individuals

- Dose : 50 mikrogram / kg
 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
Mechanisms of Action of Therapies for Immune Thrombocytopenic Purpura ( From Cines DB
et al, Immune Thrombocytopenic Purpura, In : N Engl J med 2002 ; 346 )
 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
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
DIAGNOSIS

Checking for the presence of maternal

alloantibodies
directed against the fathers platelets
Specific studies to identify the target alloantigen

DIFFERENTIAL DIAGNOSIS
Transplacental transfer of maternal antiplatelet

auto antibodies ( Maternal ITP )

Viral or bacterial infection
TREATMENT

Transfusion with antigen negatife patelets

IVIG ( 1 g / kg /24 hours, 1-2 doses until platelet

> 50.000 / mm3 )

PREVENTION
IVIG 1 g / kg /week from midgestation until near

term
Delivery should be performed by cesarean

section