A case of Glanzmann's Thrombasthenia - Diagnosed During Evaluation of Post-Surgery Bleeding.

Authors

  • Mafruha Akter Assistant Professor of Hematology & Bone Marrow Transplantation. Department of Hematology and BMT, Dhaka Medical College & Hospital. Dhaka, Bangladesh.
  • Mohammad Ibrahim Khalil Assistant professor. Department of Surgery, Dhaka Medical College & Hospital.
  • Tahmid E Rabbani Khairul Koron Indoor medical officer, Department of Surgery, Dhaka Medical College & Hospital.
  • Abu Zaffor Md Saleh Assistant registrar, Department of surgery, Dhaka Medical College & Hospital.
  • Imtiaz Faruk Department of surgery, Dhaka Medical College & Hospital.

DOI:

https://doi.org/10.3329/jss.v25i2.86013

Keywords:

Glanzmann’s thrombasthenia, GPIIb/IIIA

Abstract

Glanzmann’s thrombasthenia (GT) is a very rare inherited autosomal recessive bleeding disorder due to platelet dysfunction. Clinical presentations and severity of bleeding are heterogeneous. Bleeding pattern is usually muco-cutaneous ranging from mild bruising to potentially life threatening bleeding, although severe internal organ bleeding is rare except in trauma or post surgery without precaution. Genetic defect of GT is associated with mutations in gene encoding platelet membrane glycoprotein GPIIb/IIIA leading to lack of platelet aggregation due to reduced expression or complete absence of GPIIb/IIIA. 

Here we report a case of GT who was diagnosed first time at 20 years of age after emergency surgery without proper evaluation and precaution in a resource limited situation. However she was managed by platelet transfusion and antifibrinolytic therapy.

Journal of Surgical Sciences (2021) Vol. 25(2) : 47-51

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Published

2021-12-31

How to Cite

Akter, M., Khalil, M. I., Koron, T. E. R. K., Saleh, A. Z. M., & Faruk, I. (2021). A case of Glanzmann’s Thrombasthenia - Diagnosed During Evaluation of Post-Surgery Bleeding. Journal of Surgical Sciences, 25(2), 47–51. https://doi.org/10.3329/jss.v25i2.86013

Issue

Section

Case Reports