Cho Ray Hospital treated the following case: a 10 years-old-girl patient with Glanzmann thrombasthenia and a tumor in the right upper maxillar. The tumor's diameter was 7x8cm. It almost covered the right upper maxillar sinus, sigmoide sinus, nasal cavity and ocular cavity. The result of tumor ana pathology was -benign osteofibrosis. The patient's face had recently made a bulge in her right eye for one year. She found it difficult to perform inhaling and exhaling through the nose as well as eating. She was able to drink milk and respirate through the mouth only. The tumor must be taken out, but the hemostasis added a further complication to the surgery because she had antibody antiplatelet. Therefore, the injecting factor VII active was imperative, not platelet. Serial problems needed solving in the period of post-operation such as hemorrhage from the mouth, nose and cavity of tracheotomy. The patient was also unable to breathe easily so that she was supported by breathing machine. The results: after 53-days of treatment the wound is healed properly. Cho Ray Hospital announces this case because Glanzmann Thrombasthenia is one of the rare diseases in the world (1/1.000.000). Autosome recessive gene is in the long arm of chromosome 17. Once this gene is broken, patients don't have GP IIb/IIIa receptor. As a result of that, platelet can not aggregate and there are no clots formed. In therapy, the initial step is platelet transfusion, but gradually patients will have antibody anti-platelet. Consequently, using factor Vila is completely crucial to hemostasis. This successful case will give useful experience in treatment by factor Vila for other diseases such as Hemophilia A, B having antibody anti VIII, IX; no factor VII; Glanzmann's thrombasthenia and discoagulation after trauma.
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