Manejo de la trombastenia de Glanzmann durante el embarazo: reporte de un caso y revisión de la literatura / Management of Glanzmann's thrombasthenia during pregnancy: a case report and literature review

Resumen Objetivo: Reportar el caso de una paciente con trombastenia de Glanzmann que recibe manejo con transfusión de plaquetas con factor VII activado y realizar una revisión de la literatura referente al tratamiento y el pronóstico de esta patología durante la gestación. Método: Se presenta el caso de una paciente de 27 años con trombastenia de Glanzmann y embarazo de 33 semanas, con cesárea al término sin complicaciones. Se realizó una búsqueda en las bases de datos Medline vía PubMed, Lilacs, SciELO y ScienceDirect; se incluyeron reportes de caso, series de casos y revisiones bibliográficas hasta 2021. Resultados: Se encontraron 21 artículos, con 23 casos reportados. Los embarazos se presentaron entre la tercera y la cuarta décadas de la vida, siendo la mayoría pacientes con anticuerpos frente a antígenos plaquetarios (43,4% de los casos). El principal manejo fue con transfusión plaquetaria. Conclusiones: La trombastenia de Glanzmann durante el embarazo es infrecuente y se asocia a eventos hemorrágicos. La presencia de anticuerpos frente a antígenos plaquetarios condiciona el manejo con mayor riesgo de complicaciones perinatales. No tiene un enfoque terapéutico unificado, siendo el de elección la transfusión de plaquetas y como segunda línea el factor VII activado.

Abstract Objective: To report the case of a patient with Glanzmann's thrombasthenia who receives management with platelet transfusion with activated factor VII and a literature review regarding the treatment and prognosis of this pathology during pregnancy. Method: We present the case of a 27 year old patient with Glanzmann's thrombasthenia and a 33-week pregnancy, with a cesarean section at term without complications. Medline databases were searched via PubMed, Lilacs, SciELO and ScienceDirect; case reports, case series and bibliographic reviews were included until 2021. Results: A total of 21 articles were found, with 23 reported cases; the pregnancies occurred between the third and fourth decades of life, the majority being patients with anti-platelet antigen antibodies in 43.4% of the cases. The main management was with platelet transfusion. Conclusions: Glanzmann's thrombasthenia during pregnancy is rare and is associated with hemorrhagic events. The presence of anti-platelet antigen antibodies conditions management with a higher risk of perinatal complications. It does not have a unified therapeutic approach, with platelet transfusion being the management of choice and activated factor VII as second line.

[Glanzmann's thrombasthenia: first case descriptions in sub-saharan africa]

Constitutive hemorrhagic diseases that affect primary haemostasis are reportedly rare in sub-Saharan Africa This study arrived to report within a Congolese family live cases of Glanzmann's thromboasthenia 5 cases of the congenital form of Glanzmann's thromboasthenia were depictecd in a Congolese family. The disease was first discovered with a young student who was transferred in France who had shown a tendency to develop hemorrhages since childhood. This tendency was enhanced following abdominal surgery to treat peritonitis. Like the other 3 cases, she had a prolonged bleeding time, albeit with normal von Willebrand factor plasma values. A 7 year old girl died following appendectomy from post-surgery hemorrhages. In this young patient, platelet aggregation could be induced only by ristocetine all other conventional agonists failed. Flow cytometric analysis showed the total absence of GPIIbIIIa. The hemorrhages in the girls could be managed by cyclic administration of oestrogens and iron supplementation .Serological analysis showed this patient to be Positive for hepatitis C virus antibodies. This first description of Glanzmann's thromobo - asthenia in Blacks in sub-Saharan Africa shows the necessity of establish inter-hospital cooperationfor the improvement of the management of constitutive hemorrhagic diseases in the Hematology words

Glanzmann's thrombasthenia and pregnancy

Glanzmann's thrombasthenia is a rare congenital disorder of platelet function manifesting as defective primary haemostasis. Bleeding episodes often require platelet transfusions, and allo-immunization to donor platelets may occur. The problems of ensuring adequate haemostatic potential for delivery of an allo-immunized pregnant female with Glanzmann's thrombasthenia are presented