DISORDERS OF PRIMARY HEMOSTASIS

VASCULAR SYSTEM DISORDERS

Important lab tests for vascular system disorders:
Platelet count Normal
PT Normal
APTT Normal
Template BT Normal or abnormal
INHERITED VASCULAR DISORDERS
Hereditary hemorrhagic telangiectasia (HHT)
Ehlers-Danlos syndromes
PLATELET DISORDERS
QUANTITATIVE PLATELET DISORDERS
Thrombocytopenia
Important lab tests for thrombocytopenia:
Platelet count Decreased
PT Normal
APTT Normal
Template BT Abnormal
Due to increased destruction
By the immune system
Immune thrombocytopenic purpura (ITP)
Neonatal alloimmune thrombocytopenia (NAIT)
Heparin-induced thrombocytopenia (HIT)
By consumption
Disseminated intravascular coagulation (DIC)
Thrombotic thrombocytopenic purpura (TTP)
Hemolytic uremic syndrome (HUS)
Due to decreased production
By megakaryocyte hypoplasia syndromes
Decreased megakaryocyte proliferation
A type of aplastic anemia
Fanconi anemia
Thrombocytopenia with absent radii (TAR) syndrome
By hereditary thrombocytopenia
Wiskott-Aldrich Syndrome
Bernard-Soulier (giant platelet) syndrome
May-Hegglin anomaly

Due to increased splenic sequestration
"Big spleen" syndrome: hypersplenism
Due to dilution of blood
Multiple transfusions

Due to multiple reasons
Open-heart surgery
Thrombocytosis
Reactive (transient) thrombocytosis
Myeloproliferative disorders
QUALITATIVE PLATELET DISORDERS
Important lab tests for qualitative platelet disorders:
Platelet count Normal
PT Normal
APTT Normal
Template BT Normal or abnormal
Hereditary qualitative platelet disorders
Due to disordered platelet adhesion
Bernard-Soulier (giant platelet) Syndrome
von Willebrand Disease (vWD)
Hypergammaglobulinemia
DISORDERS OF SECONDARY HEMOSTASIS
HEREDITARY DISORDERS OF SECONDARY HEMOSTASIS
AUTOSOMAL DOMINANT
von Willebrand disease (vWD)
X-LINKED RECESSIVE
Factor VIII deficiency (hemophilia A)
Factor IX deficiency (hemophilia B)
AUTOSOMAL RECESSIVE
Factor deficiencies
Factor II (prothrombin) deficiency
Factor V deficiency
Factor VII deficiency
Factor X deficiency
Factor XI deficiency
Factor XIII deficiency
Factor XII deficiency
PK deficiency
HK deficiency
Of factor I (fibrinogen)
Afibrinogenemia
Hypofibrinogenemia
Dysfibrinogenemia
ACQUIRED DISORDERS OF SECONDARY HEMOSTASIS
Disseminated Intravascular Coagulation (DIC)
Liver disease (LD)
Hemorrhagic disease of the newborn (HDN)
Acquired inhibitors (circulating anticoagulants)
Thrombotic Disorders