Glanzmann's thrombasthenia patient information
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Glanzmann's thrombasthenia |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Omer Kamal, M.D.[2]
Overview
Symptoms of Glanzmann's thrombasthenia varies from a minor bruise to a life-threatening hemorrhage. In the hereditary type of Glanzmann's thrombasthenia GPIIb/IIIa (ITG αIIbβ3) is qualitative or quantitative disorder. The autoantibodies production is the main cause of acquired Glanzmann's thrombasthenia. Common risk factors that increase autoantibodies production in acquired Glanzmann thrombasthenia include Acute lymphoblastic leukemia, Non-Hodgkin’s lymphoma, Multiple myeloma, Hairy cell leukemia and Myelodysplastic syndrome. There is no single diagnostic study of choice for the diagnosis of Glanzmann's thrombasthenia, but it can be diagnosed based on Platelet aggregation assays which is panel of assays measuring platelet aggregation and activation in vitro. using like ADP, arachidonic acid, collagen, epinephrine, thrombin, and ristocetin.The diagnosis of Glanzmann thrombasthenia is confirmed through monoclonal antibody testing and flow cytometry. DDAVP prevents bleeding after dental extraction and minor surgery in patients with milder platelet defects. Glanzmann's thrombasthenia patients need regular dental visits and must maintain good oral hygiene because the recurrence of gingival bleeding is more in them. These patient should avoid contact sports. Estrogens, platelet transfusion, antifibrinolytic agents, and recombinant human factor VIIa are some other therapies used for treatment/prevention.
What are the symptoms of Glanzmann's thrombasthenia?
Symptoms of Glanzmann's thrombasthenia varies from a minor bruise to a life-threatening hemorrhage. It may include any of the following manifestations:
- Easily bruising (76.6%)
- Nosebleeds that do not stop easily (62.5%)
- Bleeding gums (56.4%)
- Prolonged bleeding with minor injuries (47.2%)
- Heavy menstrual bleeding
- Postpartum bleeding
- Gastrointestinal bleeding
- Heavy bleeding during and after surgery
- Bleeding into joints (rare)
What causes Glanzmann's thrombasthenia?
In the hereditary type of Glanzmann's thrombasthenia GPIIb/IIIa (ITG αIIbβ3) is qualitative or quantitative disorder. The autoantibodies production is the main cause of acquired Glanzmann's thrombastheniaIt can be produced in the of following conditions:
- Acute lymphoblastic leukemia
- Non-Hodgkin lymphoma
- Multiple myeloma
- Hairy cell leukemia
- Myelodysplastic syndrome
- Immune thrombocytopenic purpura (ITP)
- Pregnancy
- Autoimmune diseases (eg, systemic lupus erythematosus, Immune thrombocytopenia)
- Drugs : Anti-thrombotic drugs use , like abciximab, eptifibatide, and tirofiban which all antagonize αIIbβ3
- Platelet transfusions
Who is at highest risk?
Common risk factors that increase autoantibodies production in acquired Glanzmann thrombasthenia include:
- Hematologic disorders and malignancies, such as :
- Acute lymphoblastic leukemia
- Non-Hodgkin’s lymphoma
- Multiple myeloma
- Hairy cell leukemia
- Myelodysplastic syndrome
- Immune thrombocytopenic purpura (ITP)
- Autoimmune diseases such as Lupus
- Drugs : Anti-thrombotic drugs use , like abciximab, eptifibatide, and tirofiban which all antagonize αIIbβ3
- Platelet transfusions.
Diagnosis
There is no single diagnostic study of choice for the diagnosis of Glanzmann's thrombasthenia, but it can be diagnosed based on Platelet aggregation assays which is panel of assays measuring platelet aggregation and activation in vitro. using like ADP, arachidonic acid, collagen, epinephrine, thrombin, and ristocetin.The diagnosis of Glanzmann thrombasthenia is confirmed through monoclonal antibody testing and flow cytometry
When to seek urgent medical care?
Call for an appointment with your health care provider if you have a chronic disorder and you develop symptoms of Glanzmann thrombasthenia
Treatment options
The treatment of bleeding episodes in patients with glanzmann's thrombasthenia includes local measures with or without anti-fibrinolytic therapy first, followed by platelet transfusion, and rFVIIa if bleeding persists. However, The majority of cases of glanzmann's thrombasthenia are self-limited and only require supportive care. Other options include desmopressin (DDAVP) which increases in plasma, the tissue plasminogen activator (TPA),FVIII and VWF, but it has no significant effect on platelet disorders, rFVIIa: Manages bleeding in most patients with glanzmann's thrombasthenia, rituximab, bevacizumab,hematopoietic stem cell transplantation and gene therapy.
Where to find medical care for Glanzmann's thrombasthenia?
Call for an appointment with your health care provider if you have a chronic disorder and you develop symptoms of Glanzmann thrombasthenia
Prevention of Glanzmann's thrombasthenia
DDAVP prevents bleeding after dental extraction and minor surgery in patients with milder platelet defects. Glanzmann's thrombasthenia patients need regular dental visits and must maintain good oral hygiene because the recurrence of gingival bleeding is more in them. These patient should avoid contact sports. Estrogens, platelet transfusion, antifibrinolytic agents, and recombinant human factor VIIa are some other therapies used for treatment/prevention.
What to expect (Outlook/Prognosis)?
Prognosis is generally excellent with good supportive care and the mortality rate of patients with Glanzmann's thrombasthenia is relatively low
Possible complications
Common complications of include sever fatal bleeding following major surgeries , labor and delivery
Sources
https://www.wfh.org/en/sslpage.aspx?pid=658