Glanzmann's thrombasthenia

Glanzmann's thrombasthenia
Other names	Thrombasthenia of Glanzmann and Naegeli[1]
This condition is inherited in a autosomal recessive manner
Specialty	Hematology

Glanzmann's thrombasthenia is an abnormality of the

Therapy involves both preventive measures and treatment of specific bleeding episodes.[3]

• Dental hygiene lessens
  gingival bleeding^[11]
• Avoidance of antiplatelet agents such as
  NSAIDs) such as ibuprofen and naproxen
  , and anticoagulants
• Iron or folate supplementation may be necessary if excessive or prolonged bleeding has caused anemia
• Hepatitis B vaccine
• Antifibrinolytic drugs such as tranexamic acid or ε-aminocaproic acid (Amicar)
• Desmopressin (DDAVP) does not normalize the bleeding time in Glanzmann's thrombasthenia but anecdotally improves hemostasis
• Hormonal contraceptives
  to control excessive menstrual bleeding
• Topical agents such as
  fibrin sealants, polyethylene glycol
  polymers, custom dental splints
• Platelet
  alloimmunization
  )
• AryoSeven
  or NovoSeven FDA approved this drug for the treatment of the disease in July 2014.
• Hematopoietic stem cell transplantation (HSCT) for severe recurrent hemorrhages

Eponym

It is named after Eduard Glanzmann (1887-1959), the Swiss pediatrician who originally described it.^[12][13][14]

History

The subsequent studies, following Eduard Glanzmann's description of hemorrhagic symptoms and "weak platelets", demonstrated that these patients have prolonged bleeding times and their platelets failed to aggregate in response to activation. In the mid-1970s, Nurden and Caen^[15] and Phillips and colleagues^[16] discovered that thrombasthenic platelets are deficient in integrins α_IIbβ_3.

See also

• Platelet
• Coagulation
• Bernard-Soulier syndrome

References