eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders

Glanzmann Thrombasthenia: Differential Diagnoses & Workup

Author: Zonera Ashraf Ali, MD, Consulting Staff, Main Line Oncology Hematology Associates, Lankenau Cancer Center
Coauthor(s): Mark J Shumate, MD, MPH, Assistant Professor, Department of Internal Medicine, Division of Hematology/Oncology, Emory University
Contributor Information and Disclosures

Updated: Nov 20, 2008

Differential Diagnoses

Platelet Disorders
von Willebrand Disease
Wiskott-Aldrich Syndrome

Other Problems to Be Considered

Workup

Laboratory Studies

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.

Histologic Findings

The platelet morphology on peripheral blood smears is normal in patients with Glanzmann thrombasthenia.

More on Glanzmann Thrombasthenia

Overview: Glanzmann Thrombasthenia
Differential Diagnoses & Workup: Glanzmann Thrombasthenia
Treatment & Medication: Glanzmann Thrombasthenia
Follow-up: Glanzmann Thrombasthenia
References
Further Reading
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References

  1. George JN, Caen JP, Nurden AT. Glanzmann's thrombasthenia: the spectrum of clinical disease. Blood. Apr 1 1990;75(7):1383-95. [Medline]. [Full Text].

  2. Malik U, Dutcher JP, Oleksowicz L. Acquired Glanzmann's thrombasthenia associated with Hodgkin's lymphoma: a case report and review of the literature. Cancer. May 1 1998;82(9):1764-8. [Medline]. [Full Text].

  3. Nurden AT. Glanzmann thrombasthenia. Orphanet J Rare Dis. Apr 6 2006;1:10. [Medline]. [Full Text].

  4. Poon MC, Zotz R, Di Minno G, et al. Glanzmann's thrombasthenia treatment: a prospective observational registry on the use of recombinant human activated factor VII and other hemostatic agents. Semin Hematol. Jan 2006;43(1 suppl 1):S33-6. [Medline].

  5. Poon MC. Clinical use of recombinant human activated factor VII (rFVIIa) in the prevention and treatment of bleeding episodes in patients with Glanzmann's thrombasthenia. Vasc Health Risk Manag. 2007;3(5):655-64. [Medline]. [Full Text].

  6. Saxena R, Kannan M. Glanzmann's thrombasthenia: an overview. Clin Appl Thromb Hemost. Oct 16 2008;epub ahead of print. [Medline].

  7. Tholouli E, Hay CR, O'Gorman P, Makris M. Acquired Glanzmann's thrombasthenia without thrombocytopenia: a severe acquired autoimmune bleeding disorder. Br J Haematol. Oct 2004;127(2):209-13. [Medline].

  8. Ali N, Moiz B, Shaikh U, et al. Diagnostic tool for Glanzmann's thrombasthenia clinicopathologic spectrum. J Coll Physicians Surg Pak. Feb 2008;18(2):91-4. [Medline].

  9. Jin PP, Shen WZ, Yang F, et al. [Analysis of clinical features and genotype in three Chinese pedigrees with Glanzmann thrombasthenia]. Zhonghua Xue Ye Xue Za Zhi. Mar 2008;29(3):149-53. [Medline].

  10. Lombardo VT, Sottilotta G. Recombinant activated factor VII combined with desmopressin in preventing bleeding from dental extraction in a patient with Glanzmann's thrombasthenia. Clin Appl Thromb Hemost. Jan 2006;12(1):115-6. [Medline].

  11. Shen WZ, Ding QL, Jin PP, et al. A novel Pro126His beta propeller mutation in integrin alphaIIb causes Glanzmann thrombasthenia by impairing progression of pro-alphaIIbbeta3 from endoplasmic reticulum to Golgi. Blood Cells Mol Dis. Oct 29 2008;epub ahead of print. [Medline].

  12. Vuckovic SA. Glanzmann's thrombasthenia revisited. J Emerg Med. May-Jun 1996;14(3):299-303. [Medline].

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Keywords

Glanzmann thrombasthenia, thrombasthenia, Glanzmann disease, Glanzmann's thrombasthenia, platelet glycoprotein GPIIb-IIIa complex, blood platelet disorders, hemorrhagic disorders, genetic platelet disorders, platelet glycoprotein IIB, GP IIb, platelet glycoprotein IIIa, GP IIIa, GP IIa/IIIb, defective platelet aggregation, von Willebrand factor, vWF, bleeding

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Contributor Information and Disclosures

Author

Zonera Ashraf Ali, MD, Consulting Staff, Main Line Oncology Hematology Associates, Lankenau Cancer Center
Zonera Ashraf Ali, MD is a member of the following medical societies: American Society of Clinical Oncology and American Society of Hematology
Disclosure: Nothing to disclose.

Coauthor(s)

Mark J Shumate, MD, MPH, Assistant Professor, Department of Internal Medicine, Division of Hematology/Oncology, Emory University
Mark J Shumate, MD, MPH is a member of the following medical societies: American Society of Hematology
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
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, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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