Hemostatic System - general information

Normal hemostatic system

vessel wall
circulating blood platelets
blood coagulation and fibrynolysis
Platelets
disc-shape cells
arise from megakariocytes in bone marrow
1/3 of platelets are sequestrated in the spleen
other 2/3 circulate for 7-10 days
are responsible for primary hemostasis
normal platelet count : 150-450 G/l
 Bleeding Diathesis

Is caused by inherited or acquired defects of

vessel wall
platelets number and/or function
coagulation system

Is charcterised by
spontaneous bleeding
extensive bleeding after minimal trauma
 Platelets Disorder

Disorder of platelet number

thrombocytopenia
thrombocytosis
Disorder of platelet function
 Disorder of platelets function

defects of platelet adhesion

inherited: vonWillebrandts disease , Bernard-Soulier syndrome
acquired: uremia
defects of platelet aggregation
inherited: Glantzmanns thrombasthenia
acquired: dysproteinemia, drug ingestion (ticlopidin)
defects of platelet release
inherited: grey-platelet, Hermansky-Pudlak,Chediac-Higashi syndr.
acquired: cardiopulmonary bypass, myeloproliferative disorders, drugs
 Thrombocytosis
Thrombocytosis resulting from myeloproliferation
essential thrombocythemia
polycythemia vera
chronic myelogenous leukemia
myeloid metaplasia
Secondary (reactive) thrombocytosis
systemic inflammation
malignancy
iron deficiency
hemorrhage
postsplenectomy
 Thrombocytopenia (1)
Decreased marrow production of megakariocytes
congenital disorders
thrombocytopenia with absent radii (TAR)
Fanconis anemia
May-Hegglin anomaly
acquired disorders
marrow infiltration with malignant cells
marrow fibrosis
aplastic and hypoplastic anemias ( idiopathic, drugs, toxins )
deficiency states ( vitamin B12, folate, iron )
paroxysmal nocturnal hemoglobinuria
 Thrombocytopenia (2)

Splenic sequestration of circulating platelets

splenic enlargement due to tumor infiltration

splenic enlargement due to portal hypertension
 Thrombocytopenia (3)
Increased destruction of circulating platelets
congenital disorder
Wiscott-Aldrich syndrome, Bernard-Soulier syndrome
acquired disorders
nonimmune destruction
vascular prostheses, cardiac valves
DIC
hemolytic-uremic syndrome/thrombotic thrombocytopenic purpura
sepsis
immune destruction
idiopathic thrombocytopenic purpura ( ITP ) acute and chronic
drug-induced thrombocytopenia
chronic autoimmune disorders
infection (HIV)
malignancies
 Platelets Disorder - signs and symptoms

asymptomatic if platelets count > 50 G/l

onset of bleeding after trauma - immediate
site of bleeding - superficial: skin, mucous
membranes, nose, genitourinary tract
physical finding - petechiae, ecchymoses
 Idiopathic Thrombocytopenic Purpura (1)
the most common cause of isolated thrombocytopenia
autoimmune disease with antiplatelet antibodies and
shortened platelets life span
forms of ITP
acute ITP
children (90% of pediatric case of immune thrombocytopenia)
preceded by viral infection
spontaneous recovery within 4-6 weeks in 60% of patients
chronic ITP
20-40 years
women predominance F:M=3:1
 Idiopathic Thrombocytopenic Purpura (2)

Clinical features
petechiae
ecchymoses
mucose membranes bleeding
menorrhagia
rare internal, intracranial bleeding
Diagnosis
platelet count <100G/l
bleeding time - usually normal
peripheral blood smear - large platelets
bone marrow examination - normal or increased number of
megakariocytes
antibodies against Gp IIb/IIIa
shortened platelet survival
 Treatment of ITP

not necessary unless platelets count > 20G/l or there is

extensive bleeding
corticosteroids permanent responses - 30%
prednisone 1mg/kg for 4-6 weeks
splenectomy permanent responses - 60%
immunosuppresive drugs
intravenous immunoglobulins
other - danazol, antiRhD