Rav Hemo Kaplan Glanzmann Thromboasthenia

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etiology

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AR defect in GPIIb-IIIa
cannot bind to fibrinogen and will lead to abnormal aggregation

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ds

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childhood bleeding from mucosaa with normal platelet count but single, isolated platelet and lack of clumping on peripferal smear

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BT

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bleeding time is significantly prolonged.

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pt ptt platelet count

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normal

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In patients suspected with of Glanzmann thrombasthenia,

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In patients suspected with of Glanzmann thrombasthenia, examine the skin and oral mucosa for petechiae, ecchymoses, and any current bleeding.
The spleen should normally be nonpalpabl

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Differentials

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Differentials

Platelet Disorders
von Willebrand Disease
Wiskott-Aldrich Syndrome

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The workup for Glanzmann thrombasthenia may include the following:

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The workup for Glanzmann thrombasthenia may include the following:

Complete blood cell (CBC) count, prothrombin time (PT), and activated partial thromboplastin time (aPTT)
The platelet count and other coagulation tests should be normal, although the red blood cell count may be decreased due to bleeding and/or concomitant iron deficiency.
The bleeding times should be prolonged.
The Platelet Function Analyzer 100 (PFA-100) is a device that may be helpful in determining the cause of bleeding diathesis. Patients with Glanzmann thrombasthenia fail to plug the collagen-based filter.
Flow cytometry and monoclonal antibodies confirm the diagnosis of Glanzmann thrombasthenia.
Platelet aggregation studies
The primary platelet aggregation response to platelet agonists such as adenosine diphosphate (ADP), epinephrine, and collagen are decreased, whereas the response to ristocetin is normal.
If the secondary platelet aggregation response is abnormal, suspect a platelet storage pool defect or an abnormality in platelet signal transduction.

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Patients with Glanzmann thrombasthenia who are bleeding require

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Patients with Glanzmann thrombasthenia who are bleeding require platelet transfusion.
Because patients are likely to require multiple transfusions during their lifetime, take care to avoid platelet alloimmunization.
Prevention is best accomplished by using leukocyte-depleted blood products. Leukocyte depletion can be accomplished with mechanical filtration. Only filtered blood products should be given.
Use of platelets matched via human leukocyte antigen (HLA) is a further attempt to prevent platelet alloimmunization.
Patients should be vaccinated for hepatitis B due to the infectious risks associated with multiple transfusions
Avoid medications that affect platelet function, such as aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs).
Consider oral contraceptives to control menorrhagia.
Recombinant human-activated factor VII (rFVII) has been used in patients with antibodies to GP IIb/IIIa and/or HLA that render transfusions ineffective. The exact dosing of rFVII for Glanzmann thrombasthenia has not been determined.

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Surgical Care

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Surgical Care

For patients with Glanzmann thrombasthenia who require surgical intervention:

To prevent excessive bleeding during surgery, platelets should be transfused preoperatively.
Further platelet transfusions are considered, based on maintaining hemostasis.
rFVIIa may be useful for preventing or controlling bleeding.