The hemorrhagic diatheses constitute a special group

The Passovoy defect: further characterization of a hereditary hemorrhagic diathesis

hemorrhagic diasthesis : Über dieses Forum

A case of atrial septal defect with idiopathic thrombocytopenia was successfully operated with the aid of heart-lung machine. The operation was performed under the administration of platelet rich plasma, fresh blood and adrenocorticosteroids before, during and after the operation .With the aid of these treatments, no severe hemorrhagic diasthesis was encountered.

Related to diatheses: Hemorrhagic diathesis, ..

usually resulting in hemorrhagic diasthesis.

Since Tuohy's first description in 1920, more than 50 cases fullfilling the above criteria have been reported worldwide, and 4 cases in Korea. Several authors have demonstrated the defect of intracellualr killing in the mature neutrophil and this finding correlates well with the very high incidence of fatal infection, The hemorrhagic diasthesis in CNL is caused by functional abnormality of the platelet. This disease has tendency to transform to blastic crisis and acute leukemia as in other myeloproliferative disease, but characteristically shows frequent coexistence with multiple myeloma. Until now , the therapeutic trials in CNL have been disappointing. Hydroxyrea and busulfan can control hyperleukocytosis. On the basis of functional defect in neutrophil, alpha-2b-interferon has been tried and several reports have demonstrated the clinical and functional effect of interferon on CNL.

The operation was performed under the administration of platelet rich plasma, fresh blood and adrenocorticosteroids before, during and after the operation .With the aid of these treatments, no severe hemorrhagic diasthesis was encountered.