Glanzmann's disease: clinical elements and therapeutic behavior

Abstract

Introduction: Glanzmann's disease is a rare disorder, with a worldwide distribution that predominates in the Asian region; it has specific symptoms and emergent treatment. Objective: to describe the clinical elements and the therapeutic behavior of Glanzmann's disease. Method: a bibliographic review was carried out in the databases SciELO, Scopus, ClinicalKey and PubMed. The combination of terms was used as a search strategy, using the name of the disease, clinic and treatment. 21 references were selected. Development: there are three subtypes of Glanzmann's thrombasthenia. The clinical manifestations are variable and include mucocutaneous and gastrointestinal hemorrhages, presence of hemarthrosis, intracranial hemorrhage, and visceral hematoma. Prophylaxis is recommended before major surgeries and avoiding the use of drugs that affect platelet function such as non-steroidal anti-inflammatory drugs and aspirin. The only curative measure for patients with recurrent bleeding episodes or who are refractory to platelet transfusions is allogeneic hematopoietic cell transplantation. Conclusions: the clinical method that includes the search for history and a rigorous physical examination, accompanied by complementary tests, plays an important role in the diagnosis of rhombobastenia. In this way, therapeutics focuses on controlling acute symptoms and platelet transfusions, with several options such as gene therapy still under study; all in order to improve the quality of life and extend it.

Keywords

• trombastenia
• hemorragia
• agregación plaquetaria
• trombastenia de glanzmann.