Platelet Glycoproteins in Inherited Thrombocytopathy: Association With Aggregation Studies and Bleeding Severity

NCT03648190 | Unknown

• 1 other identifier: 17200237
• Study Type: observational
• Target: 50
• Locations: 1 country
• Sites: 1

Brief Summary

Disorders of platelet function are characterized by variable mucocutaneous bleeding manifestations and excessive hemorrhage following surgical procedures or trauma. Generally, most patients have mild to moderate bleeding manifestations with a prolonged bleeding time. Platelet aggregation and secretion studies using platelet-rich plasma (PRP) provide evidence for platelet dysfunction but are neither predictive of severity of clinical manifestations nor the molecular mechanisms. Glanzmann's thrombasthenia (GT) is a rare autosomal recessive genetic bleeding syndrome characterized by defects in platelet aggregometry. The clinical phenotype of patients with GT is variable. Some suffer from severe bleeding, while others have only mild bleeding. Some studies found bleeding severity in GT was influenced by the abundance and functioning of platelet receptors involved in aggregation and adhesion. In addition to a complete medical history, a GT diagnosis requires a comprehensive laboratory workup, including platelet aggregation analysis, and a confirmation by flowcytometry or western blotting with monoclonal antibodies that recognize the GPIIb/IIIa complex. Platelet flow cytometry is an emerging tool in diagnostic and therapeutic hematology. It is eminently suited to study the expression of platelet surface receptors both qualitatively as well as quantitatively. Aim of the study:-

• Determine the role of flowcytometry as a quantitative measurement tool of platelets surface glycoproteins in patients with inherited thrombocytopathies and its correlation with bleeding severity of these patients.
• To compare the efficacy, advantages and disadvantages between platelets flowcytometry and aggregometer in diagnosing various inherited thrombocytopathies.

Conditions

Bleeding; Platelet Dysfunction

Keywords

GT; CD

Outcome Measures

Primary Outcomes (1)

• Establishment of a protocol for Flowcytometry device to be used in routinely diagnosing cases with inherited platelets function defects.

  Determine the ability of flowcytometry to assay (determine the levels of expression of each surface marker in percent) platelets surface glycoproteins (CD 41, CD 61 and CD 42b) using BD FACSCalibur flowcytometry instrument in patients with inherited thrombocytopathies and its correlation with bleeding severity of these patients with establishment of a protocol for it to be used in routinely diagnosing these cases.

  6 Weeks

Study Arms (2)

Inherited qualitative platelets defect

Clinical manifestations in the form of mucocutaneous bleeding or hemorrhage. Bleeding patients with acquired bleeding disorders, coagulation defects, and those on antiplatelet drugs will be excluded from the study.

Diagnostic Test: Flowcytometry analysis of platelets surface receptors

Control

Normal healthy participants, with no manifestations of bleeding disorders.

Diagnostic Test: Flowcytometry analysis of platelets surface receptors

Interventions

Flowcytometry analysis of platelets surface receptors DIAGNOSTIC_TEST

BD FACSCalibur flowcytometry instrument. Platelet flow cytometry is an emerging tool in diagnostic and therapeutic hematology. It is eminently suited to study the expression of platelet surface receptors both qualitatively as well as quantitatively. Flow cytometry rapidly measures the specific characteristics of a large number of cells in suspension. Typically, the cells are labeled with fluorescently conjugated monoclonal antibodies (mAbs).

Also known as: Platelets aggregation tests by aggregometer

Control; Inherited qualitative platelets defect

Eligibility Criteria

• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

Bleeding patients suffering from inherited qualitative platelets defect, with the exclusion of coagulation defects, acquired bleeding disorders and anti-platelet drugs intake. Both male and females are eligible with no age limits.

You may qualify if:

• Bleeding patients.

You may not qualify if:

• Coagulation defects, and those on anti-platelet drugs.

Sponsors & Collaborators

• Assiut University: lead

Study Sites (1)

Assiut University Hospital

Asyut, 71515, Egypt

RECRUITING

Related Publications (3)

• Zhou L, Jiang M, Shen H, You T, Ding Z, Cui Q, Ma Z, Yang F, Xie Z, Shi H, Su J, Cao L, Lin J, Yin J, Dai L, Wang H, Wang Z, Yu Z, Ruan C, Xia L. Clinical and molecular insights into Glanzmann's thrombasthenia in China. Clin Genet. 2018 Aug;94(2):213-220. doi: 10.1111/cge.13366. Epub 2018 May 22.

  PMID: 29675921 BACKGROUND

• Harrison P. Assessment of platelet function in the laboratory. Hamostaseologie. 2009 Jan;29(1):25-31.

  PMID: 19151842 BACKGROUND

• Saboor M, Moinuddin M, Ilyas S. New horizons in platelets flow cytometry. Malays J Med Sci. 2013 Mar;20(2):62-6.

  PMID: 23983579 BACKGROUND

MeSH Terms

Conditions

Hemorrhage

Condition Hierarchy (Ancestors)

Pathologic Processes; Pathological Conditions, Signs and Symptoms

Study Officials

• Hanan Galal, MD

  Assiut University

  PRINCIPAL INVESTIGATOR

• Madleen Adel, MD

  Assiut University

  STUDY CHAIR

• Eman Nasr-Eldin, MD

  Assiut University

  STUDY CHAIR

• Mohammed Ashraf, M.Sc

  Assiut University

  STUDY DIRECTOR

Central Study Contacts

Mohammed Ashraf, M.Sc

CONTACT

+201002867611; M1111a1111@yahoo.com

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Assistant Lecturer

Study Record Dates

• First Submitted: August 21, 2018
• First Posted: August 27, 2018
• Study Start: December 1, 2018
• Primary Completion: December 30, 2019
• Study Completion: August 30, 2020
• Last Updated: August 28, 2018

Record last verified: 2018-08

Locations

EG (1)