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Glanzmann Thrombasthenia 
DR. VI JAY DIHORA
Normal Physiology-Production and Number 
of Platelet 
Platelets are normally made in the bone marrow from 
progenitor cells known as megakaryocytes. 
Normal platelet lifespan is 10 d. Every day, 1/10 of 
platelet pool is replenished. 
Normal platelet count is between 150,000 and 
450,000/mm3
Platelet Production 
Platelet production is regulated by thrombopoietin 
(TPO). 
Thrombopoietin binds to the surface of 
megakaryocytes and platelets-- 
 only free TPO stimulates platelet production 
 Therefore, platelet mass negatively regulates free TPO and 
further platelet production
Platelet Plug Formation 
Adhesion - Platelets stick to injured vessel wall. 
Aggregation - Platelets stick to each other via 
fibrinogen bridges. 
Secretion - Platelets release granular contents and 
potentiate clotting
STRUTURE 
Mucopolysacch. 
coat 
Granules 
Dense core 
granules 
Mucopolysacch. Coat: Glycoprotein content which are 
important for interaction of platelets with each other or 
aggregating agents. 
-  Granules : express the adhesion molecule P-selectin 
and CD63. these are transfereed to the membran after 
synthesis 
- Dense core : contain ADP and ATP, ionazed calcium 
,Histamine and Serotonine
PLATELETS ADHESION 
Adhesion of platelet to subendothelial collagen. 
Dependent on the VW factor (Von Willebrand 
part of Fact VIII). Also dependent on 
glyoproteins.
Membrane Phospholipid 
 
Arachidonic acid 
 Cyclo-oxygenase 
Thromboxane synthetase  
Thromboxane A2 
Thromboxane A2: Potentiase aggregation 
and vasoconstrictor. 
Aggregation: Release ADP+thromboxane 
A2 aggregation. This is followed by more 
secration secondary aggregation. 
platelet mass or plug.
Primary Hemostasis: Platelet adhesion 
GPIb 
EC 
Sub Endo 
glycoprotein Ib (GPIb) – 
platelet receptor for VWF
Primary Hemostasis: Platelet 
aggregation 
EC EC 
Fibrinogen 
GPIIbIIIa 
GPIIbIIIa – platelet receptor for 
fibrinogen
Classification of platelet disorders 
Quantitative disorders 
Production 
– Reduced 
Infiltration (e.g. tumor) 
Aplasia (e.g. chemicals) 
Congenital (e.g. Wisskot aldrich syn) 
– Ineffective 
Megaloblastic anemia, 
myelodysplasia, 
Destruction 
– Immune 
Autoantibody e.g. ITP 
Alloantibody 
– HIT 
– Consumption 
DIC 
TTP 
Mechanical 
Hemodilution 
Qualitative disorders 
• Inherited 
– Bernard-Soulier 
– Glanzmann’s 
– Storage pool disease 
(ChediakHigashi, WiscottAldrich, Gray 
Platelet Syndrome) 
• Acquired 
– Drugs (e.g. ASA) 
– Uremia, Post-bypass 
– Primary marrow disorders; MDS, 
Dysproteinemias
Glanzmann Thrombasthenia 
Is inherited in an autosomal recessive manner. 
The genes of both of these proteins are on 
chromosome 17. 
Different genetic mutations of either GP IIb or 
IIIa genes result in a heterogeneity of 
thrombasthenia phenotype. 
Carrier detection in GT is important to control the 
disease in family members. 
Can be acquired as an autoimmune disorder
PATHOGENESIS 
Platelet glycoprotein IIb/IIIa (GP IIb/IIIa) 
complex is deficient or present but 
dysfunctional. 
Defect in the GP IIb/IIIa complex leads to 
defective platelet aggregation and 
subsequent bleeding. 
Aggregation of PLTs occurs in response to 
ristocetin, but not to other agonists 
such as ADP, thrombin, collagen or 
epinephrine.
Severity 
Glanzmann Thrombasthenia has three categories of 
severity, depending on the importance of the platelet 
deficiency in Glycoprotein IIb/IIIa. 
• Type 1 (Severe): A level less than 5% of 
normal 
• Type II (Less severe): A level between 5% 
and 20% of normal 
• Type III (Least severe): A variant of 
Thrombasthenia with levels of more than 
50% of normal, but with major abnormalities 
in the way platelets aggregate
Symptoms 
purpura 
Menorrhagia 
Mucosal bleeding 
Brain hemorrhages 
epistaxis 
gingival bleeding 
gastrointestinal bleeding 
postpartum bleeding 
increased bleeding post-operatively
DIAGNOSIS 
Normal PLT count and morphology. 
Greatly prolonged bleeding time. 
Absence of PLT aggregation in response 
to ADP,collagen,epinephrine or thrombin 
(Platelet aggregation test) 
Flow cytometry (CD 41,CD 61). 
Studies of GP IIb/IIIa receptors on the PLT 
membrane.
Aggregation of thrombocytes (platelets). Platelet rich human blood 
plasma (left vial) is a turbid liquid. Upon addition of ADP, platelets 
are activated and start to aggregate, forming white flakes (right vial)
Treatment 
- Dental hygiene lessens gingival bleeding 
- Avoidance of antiplatelet agents such as aspirin and other anti-inflammatory 
drugs (NSAIDs) such as ibuprofen and naproxen, and 
anticoagulants 
- Iron or folate supplementation 
- Antifibrinolytic drugs such as tranexamic acid or ε-aminocaproic 
acid 
- Desmopressin (DDAVP) does not normalize the bleeding time in 
Glanzmann's thrombasthenia but anecdotally improves hemostasis 
- Hormonal contraceptives to control excessive menstrual bleeding 
- Platelet transfusions (only if bleeding is severe; risk of 
platelet alloimmunization) 
- Recombinant factor VIIa 
- Hematopoietic stem cell transplantation (HSCT) for severe 
recurrent hemorrhages

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Glanzmann thrombasthenia

  • 2. Normal Physiology-Production and Number of Platelet Platelets are normally made in the bone marrow from progenitor cells known as megakaryocytes. Normal platelet lifespan is 10 d. Every day, 1/10 of platelet pool is replenished. Normal platelet count is between 150,000 and 450,000/mm3
  • 3. Platelet Production Platelet production is regulated by thrombopoietin (TPO). Thrombopoietin binds to the surface of megakaryocytes and platelets--  only free TPO stimulates platelet production  Therefore, platelet mass negatively regulates free TPO and further platelet production
  • 4. Platelet Plug Formation Adhesion - Platelets stick to injured vessel wall. Aggregation - Platelets stick to each other via fibrinogen bridges. Secretion - Platelets release granular contents and potentiate clotting
  • 5. STRUTURE Mucopolysacch. coat Granules Dense core granules Mucopolysacch. Coat: Glycoprotein content which are important for interaction of platelets with each other or aggregating agents. -  Granules : express the adhesion molecule P-selectin and CD63. these are transfereed to the membran after synthesis - Dense core : contain ADP and ATP, ionazed calcium ,Histamine and Serotonine
  • 6. PLATELETS ADHESION Adhesion of platelet to subendothelial collagen. Dependent on the VW factor (Von Willebrand part of Fact VIII). Also dependent on glyoproteins.
  • 7.
  • 8. Membrane Phospholipid  Arachidonic acid  Cyclo-oxygenase Thromboxane synthetase  Thromboxane A2 Thromboxane A2: Potentiase aggregation and vasoconstrictor. Aggregation: Release ADP+thromboxane A2 aggregation. This is followed by more secration secondary aggregation. platelet mass or plug.
  • 9. Primary Hemostasis: Platelet adhesion GPIb EC Sub Endo glycoprotein Ib (GPIb) – platelet receptor for VWF
  • 10. Primary Hemostasis: Platelet aggregation EC EC Fibrinogen GPIIbIIIa GPIIbIIIa – platelet receptor for fibrinogen
  • 11. Classification of platelet disorders Quantitative disorders Production – Reduced Infiltration (e.g. tumor) Aplasia (e.g. chemicals) Congenital (e.g. Wisskot aldrich syn) – Ineffective Megaloblastic anemia, myelodysplasia, Destruction – Immune Autoantibody e.g. ITP Alloantibody – HIT – Consumption DIC TTP Mechanical Hemodilution Qualitative disorders • Inherited – Bernard-Soulier – Glanzmann’s – Storage pool disease (ChediakHigashi, WiscottAldrich, Gray Platelet Syndrome) • Acquired – Drugs (e.g. ASA) – Uremia, Post-bypass – Primary marrow disorders; MDS, Dysproteinemias
  • 12.
  • 13. Glanzmann Thrombasthenia Is inherited in an autosomal recessive manner. The genes of both of these proteins are on chromosome 17. Different genetic mutations of either GP IIb or IIIa genes result in a heterogeneity of thrombasthenia phenotype. Carrier detection in GT is important to control the disease in family members. Can be acquired as an autoimmune disorder
  • 14. PATHOGENESIS Platelet glycoprotein IIb/IIIa (GP IIb/IIIa) complex is deficient or present but dysfunctional. Defect in the GP IIb/IIIa complex leads to defective platelet aggregation and subsequent bleeding. Aggregation of PLTs occurs in response to ristocetin, but not to other agonists such as ADP, thrombin, collagen or epinephrine.
  • 15. Severity Glanzmann Thrombasthenia has three categories of severity, depending on the importance of the platelet deficiency in Glycoprotein IIb/IIIa. • Type 1 (Severe): A level less than 5% of normal • Type II (Less severe): A level between 5% and 20% of normal • Type III (Least severe): A variant of Thrombasthenia with levels of more than 50% of normal, but with major abnormalities in the way platelets aggregate
  • 16. Symptoms purpura Menorrhagia Mucosal bleeding Brain hemorrhages epistaxis gingival bleeding gastrointestinal bleeding postpartum bleeding increased bleeding post-operatively
  • 17. DIAGNOSIS Normal PLT count and morphology. Greatly prolonged bleeding time. Absence of PLT aggregation in response to ADP,collagen,epinephrine or thrombin (Platelet aggregation test) Flow cytometry (CD 41,CD 61). Studies of GP IIb/IIIa receptors on the PLT membrane.
  • 18. Aggregation of thrombocytes (platelets). Platelet rich human blood plasma (left vial) is a turbid liquid. Upon addition of ADP, platelets are activated and start to aggregate, forming white flakes (right vial)
  • 19. Treatment - Dental hygiene lessens gingival bleeding - Avoidance of antiplatelet agents such as aspirin and other anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen, and anticoagulants - Iron or folate supplementation - Antifibrinolytic drugs such as tranexamic acid or ε-aminocaproic acid - Desmopressin (DDAVP) does not normalize the bleeding time in Glanzmann's thrombasthenia but anecdotally improves hemostasis - Hormonal contraceptives to control excessive menstrual bleeding - Platelet transfusions (only if bleeding is severe; risk of platelet alloimmunization) - Recombinant factor VIIa - Hematopoietic stem cell transplantation (HSCT) for severe recurrent hemorrhages