Bernard-Soulier syndrome or idiopathic thrombocytopenic purpura: A case series

Bernard-Soulier syndrome.

Bernard-Soulier syndrome (BSS) is a rare autosomal bleeding disorder characterized clinically by prolonged skin bleeding time, normal clot retraction and thrombocytopenia with large and morphologically abnormal platelets, and biochemically by the absence of platelet membrane glycoproteins (GP) Ib, V and IX. GP Ib and GP IX exist in the platelet membrane as a heterodimer complex which acts as th...

Borderline Thrombocytopenia or Mild Idiopathic Thrombocytopenic Purpura?

Khush Mittal I read the paper by Chi et al. with interest. The authors found a greater hypoxia response gene expression in carcinomas than stromal cells grown in vitro, but were not sure of the underlying explanation (see Discussion in [1]). The underlying explanation may be the difference in mitotic activity and hence metabolic activity between these two cell types, such that hypoxia may be pr...

Bernard Soulier Syndrome: A Genetic Bleeding Disorder

Epidemiology of Idiopathic Thrombocytopenic Purpura in Children

Abstract Background Immune thrombocytopenic purpura (ITP) is a common autoimmune bleeding condition in children that is characterized by a decrease in the platelet count. The aims of this study were to define epidemiologic features of patients with primary ITP who were admitted to Mofid Children's Hospital, Tehran, Iran, in a 5-year period. Methods We retrospectively studied the records of ...

Idiopathic thrombocytopenic purpura.

Idiopathic thrombocytopenic purpura in children usually a self limiting disorder. It may follow a viral infection or immunisation and is caused by an inappropriate response of the immune system. About 20-30% of children will fail to remit over six months (chronic idiopathic thrombocytopenic purpura). This is more likely in older children, especially girls. The disease is reviewed with reference...

Bernard-Soulier syndrome (Hemorrhagiparous thrombocytic dystrophy)

Bernard-Soulier syndrome (BSS), also known as Hemorrhagiparous thrombocytic dystrophy, is a hereditary bleeding disorder affecting the megakaryocyte/platelet lineage and characterized by bleeding tendency, giant blood platelets and low platelet counts. This syndrome is extremely rare as only approximately 100 cases have been reported in the literature. Clinical manifestations usually include pu...