Laboratory Studies

The workup for Glanzmann thrombasthenia may include the following:

Complete blood cell (CBC) count
Peripheral blood smear
Prothrombin time (PT)
Activated partial thromboplastin time (aPTT)
Platelet Function Analyzer 100 (PFA-100) study
Flow cytometry
Monoclonal antibody assays
Platelet aggregation studies

Results are as follows:

The platelet count and coagulation tests should be normal.
The red blood cell count may be decreased due to bleeding and/or concomitant iron deficiency.
The platelet morphology on peripheral blood smears is normal.
The bleeding times should be prolonged.
On PFA-100 testing in patients with Glanzmann thrombasthenia, the platelets fail to plug the collagen-based filter.
Flow cytometry and monoclonal antibodies confirm the diagnosis of Glanzmann thrombasthenia.
On 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.

Treatment & Management

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Author

Zonera Ashraf Ali, MBBS Consulting Staff, Main Line Oncology Hematology Associates, Lankenau Cancer Center

Zonera Ashraf Ali, MBBS is a member of the following medical societies: American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, SWOG

Disclosure: Partner received none from No financial interests for none.

Chief Editor

Srikanth Nagalla, MD, MS, FACP Chief of Benign Hematology, Miami Cancer Institute, Baptist Health South Florida; Clinical Professor of Medicine, Florida International University, Herbert Wertheim College of Medicine

Srikanth Nagalla, MD, MS, FACP is a member of the following medical societies: American Society of Hematology, Association of Specialty Professors

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Alexion; Alnylam; Kedrion; Sanofi; Dova; Apellis; Pharmacosmos<br/>Serve(d) as a speaker or a member of a speakers bureau for: Sobi; Sanofi; Rigel.

Acknowledgements

Wadie F Bahou, MD Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook

Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

Mark J Shumate, MD, MPH Assistant Professor, Department of Internal Medicine, Division of Hematology/Oncology, Emory University School of Medicine

Mark J Shumate, MD, MPH is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

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