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Biomedical subjects

E Mayne

Publications and source records attributed to E Mayne.

7 recordsLinked to original sources

Octreotide in the treatment of gastrointestinal bleeding caused by angiodysplasia in two patients with von Willebrand's disease.

Two cases of von Willebrand's disease and angiodysplasia with intractable gastrointestinal bleeding are presented. Replacement therapy with cryoprecipitate and variable purity von Willebrand factor (VWF) was ineffective, as were other treatments including steroids, immunoglobulin and hormonal replacement. Both patients required massive blood transfusion and product support. The efficacy of somatostatin and an analogue is described. In one patient, we observed a rise in von Willebrand factor activity after octreotide infusion.

Aged↗

Orthotopic liver transplantation for hepatitis C infection: the best Christmas present?

We report the case of a 60-year-old man with mild Christmas disease, Factor IX 10% of normal, who developed chronic hepatitis C infection after receiving coagulation factor concentrates. Subsequently he developed encephalopathy and liver failure and was referred for liver transplantation. Following transplantation, Factor IX levels rapidly normalised and have remained so, representing a phenotypic cure of his Christmas disease.

Blood Coagulation Factors↗

Malignant lymphoma in congenital dyserythropoietic anaemia type III.

A 60 year old woman with congenital dyserythropoietic anaemia (CDA) type III developed a malignant T cell lymphoma with cutaneous and widespread nodal involvement. Bone marrow aspirates showed erythroid hyperplasia and dyserythropoiesis with multinucleate erythroblasts and gigantoblasts, in keeping with CDA type III. Electron microscopy showed multinucleate erythroblasts with notably irregular nuclear outlines and intranuclear clefts. The development of malignant lymphoma in this patient, together with a documented high prevalence of monoclonal gammopathy and multiple myeloma and a single case of Hodgkin's disease, may indicate an increased incidence of lymphoproliferative disease in CDA type III.

Anemia, Dyserythropoietic, Congenital↗