Diagnosis of Bernard Soulier Syndrome and Glanzmann’s Thrombasthenia in Iraqi Patients

Authors

  • Lina Mussa Kadhim
  • Muthana Ibrahim Malak
  • Safa A Faraj

DOI:

https://doi.org/10.37506/ijfmt.v15i2.15041

Keywords:

Inherited platelet function disorders, Bernard–Soulier syndrome, Glanzmann Thrombasthenia

Abstract

Background: Bernard–Soulier syndrome (BSS) and Glanzmann Thrombasthenia (GT) are rare inherited
platelet function disorders, defined by a permanent history of mucocutaneous bleeding. The objective of this
project was to identify biological and clinical characteristics of BSS and GT patients. Methodology: This
study included 52 patients with bleeding disorders, the clinical, haematological and demographic features
of patients were determined and the level of GpIIb/IIIa and GPIb/IX was assessed. Results: From a total of
52 patient, 20 were diagnosis with BSS (9 females and 11 males), their age range from 6 to 42 year and 23
diagnoses with GT (10 females and 13 males), their age range from 4 to 50 year. Epistaxis, easy bruising,
menorrhagia and ecchymosis were the most frequent symptoms. Prolonged bleeding time (BT), normal PT
and PTT were seen in all cases. Variable thrombocytopenia, large platelets with decreased GPIb/IX level
were seen in BSS patients, while the level of GPIIb/IIIa was decreased in GT cases. Conclusion: Since
BSS and GT are infrequent disorders diagnosis may be postponed, both diseases are combined with crucial
bleeding tendency, therefore earlier detection would have been important for patients management.

Author Biographies

Lina Mussa Kadhim

Assistant Lecturer

Muthana Ibrahim Malak

Assistant Professor at Wasit University, College of Science, Biology Department

Safa A Faraj

Assistant Professor at Wasit University, College of Medicine, MD Pediatrics Hema-Oncologist

Published

2021-03-24

How to Cite

Lina Mussa Kadhim, Muthana Ibrahim Malak, & Safa A Faraj. (2021). Diagnosis of Bernard Soulier Syndrome and Glanzmann’s Thrombasthenia in Iraqi Patients. Indian Journal of Forensic Medicine & Toxicology, 15(2), 4271-4277. https://doi.org/10.37506/ijfmt.v15i2.15041