Antiplatelet antibodies in cases of Glanzmann's thrombasthenia with and without a history of multiple platelet transfusion.

Antiplatelet antibodies are known to be present in a wide spectrum of patients, which include chronic Idiopathic Thrombocytopenic Purpura (ITP), infections, etc., including Glanzmann's thrombasthenia (GT) patients who receive multiple platelet transfusions. The presence of natural antibodies to platelet receptors is not studied in cases of GT. We studied the antiplatelet antibodies in 23 patients with GT, 15 of which had received multiple transfusions and eight that had not received transfusions, along with 50 cases of chronic ITP. The prevalence and specificity of platelet-bound antibodies were detected by inhibition assays using O-group platelets on flow cytometry. The mean antiplatelet antibodies in 15 patients of GT who had not received transfusions and eight patients with multiple transfusions was 8427 + 2131.88 and 9038 + 2856 antibodies/platelet, respectively, while in case of the 50 ITP patients studied, it was 22166 + 5616 antibodies/platelet (Normal Range 1500–3200 antibodies/platelet). We conclude that GT patients who have not received transfusions may develop antiplatelet antibodies to the missing/abnormal receptor. Whether this is due to a molecular mimicry or due to some other mechanism needs to be explored.

Glanzmann's thrombasthenia associated with HBsAg-positive child: a case report.

Glanzmann's thrombasthenia is a rare hemorrhagic disorder characterized by prolonged bleeding time and diminished clot retraction. The disease is marked by frequent mucocutaneous hemorrhage which is mainly due to qualitative defects of platelets. A case of a 14-year-old HBsAg-positive adolescent male with Glanzmann's thrombasthenia has been presented.

Doenças hemorrágicas no atendimento odontológico em crianças / Hemorrhagic diseases in pediatric dentistry

O atendimento odontológico a pacientes com doenças hemorrágicas confere grande responsabilidade ao profissional, para a identificaçäo do problema e adequada atuaçäo, numa atitude multidisciplinar com os demais membros da equipe de saúde. Este aspecto é bem enfatizado na prática da Odontopediatria, em que o bebê ou criança pode estabelecer um primeiro contato sem o diagnóstico prévio de qualquer distúrbio na hemostasia. Por esta razäo, as autoras efetuaram uma revisäo da literatura sobre as patologias mais frequentes observadas, subordinadas ao tema abordado, com base em levantamento piloto das crianças atendidas no setor de Odontologia da Fundaçäo Hemope

Manejo preoperatorio de una paciente con trastorno de la hemostasia / Preoperative management of a patient with disorders of the hemostasis

Se presenta el caso de un paciente portador de la enfermedad de Glanzmann que ingresó al servicio de emergencias del Hospital Interzonal General de Agudos Gral. San Martin de La Plata y fue intervenido quirúrgicamente con diagnóstico de apendicitis aguda. Se hace especial referencia al manejo y preparación clínico-hematológicos antes de la operación, como así tambien a los detalles de la intervención quirúrgica y al seguimiento postoperatorio. Se hacen consideraciones bibliográficas, clínicas y quirúrgicas con el fin de aportar este curioso caso a la casuística.

Glanzmann's thrombasthenia.

During January 1981 to June 1991, 20 patients from 16 unrelated families were detected to have Glanzmann's thrombasthenia (GT). Twelve families (75%) had history of consanguinity, with 6 first cousins and 3 uncle-niece marriages; of these 7 were Muslims, 6 Hindus and 3 Christians. There were 12 girls and 8 boys; the mean age at diagnosis was 7.05 +/- 6.03 yr (range 1 day-22 yr). All cases had initial bleeding prior to the age of 5 yr with the mean age at the initial episode of bleeding being 2.21 +/- 1.34 yr (range 1 day-5 yr). Common pattern of bleeding included epistaxis, gingival bleeding, post-traumatic bruises, menorrhagia, gastrointestinal (2 cases), post-operative (2 cases) and spontaneous bleeding (2 cases). No patient showed hemarthrosis, intracranial bleeding or hemoptysis. Menorrhagia was a serious problem necessitating repeated transfusions and hormonal therapy. Twelve cases (60%) required 1-120 units of blood transfusions while five received platelet concentrates.

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

Reacción de liberación en tromboastenia de Glanzmann / Release reaction in Glanzmann's thrombasthenia

Se estudiaron siete pacientes con tromboastenia de Glanzmann. Se realizó agregación plaquetaria con reacción de liberación en un agregómetro Lumi. No se observó agregación con ADP, adrenalina o colágeno. El ácido araquidónico indujo una agregación de sólo 14,9%. Con ristocetina y con factor VIII bovino la aglutinación fue marcadamente disminuida. La liberación de ATP estuvo ausente con todos los agentes agregantes excepto con ácido araquidónico que provocó una liberación normal. Se realizó curva dosis respuesta con análogo de PGH2. Con dosis de 1 micronM a 100 micronM sólo se obtuvo una mínima agregación mientras que la liberación de ATP fue normal. Los resultados confirmarían la independencia de los mecanismos de agregación y liberación. La liberación de ATP inducida por ácido araquidónico o análogo de endoperóxido no parece requerir la exposición y fijación del fibrinógeno a su receptor