Résumé
Bleeding is a common manifestation of inherited and acquired disorders of haemostasis. Acquired disorders of haemostasis can be of varied etiology like liver disease, DIC, haemorrhagic disease of newborn and inhibitors to coagulation factors. Inhibitors to coagulation factors are an unusual cause of bleeding which can be superimposed on inherited factor deficiencies or sometimes resembles them. The clinical and haematological profile to two cases of factor VIII inhibitors are being presented here, one of which was a known haemophiliac receiving factor VIII therapy and another was a elderly lady with no other apparent underlying disorder. Hence any case of factor VIII deficiency who becomes refractory to factor VIII replacement therapy or those who are detected to have factor deficiency late in life should be investigated for inhibitors.
Sujets)
Adulte , Sujet âgé , Tests de coagulation sanguine , Facteur VIII/antagonistes et inhibiteurs , Femelle , Hémophilie A/sang , Humains , Mâle , Temps partiel de thromboplastineRésumé
Haemophilia in a female is very rare. We report a case of haemophilia in a female with a male sex chromosome pattern.
Sujets)
Adulte , Syndrome d'insensibilité aux androgènes/génétique , Femelle , Hémarthrose/génétique , Hémophilie A/sang , Humains , Caryotypage , Mâle , Pedigree , PhénotypeRésumé
Wolman's disease is a rare autosomal recessive lysosomal storage disorder. We report a case, which we identified with foamy histiocytes in bone marrow and adrenal calcification in radiological imaging. The diagnosis can be made on minimal investigation when clinically suspected. But cytogenetic study is required to substantiate the diagnosis further.
Sujets)
Maladies des surrénales/anatomopathologie , Calcinose/anatomopathologie , Femelle , Humains , Nourrisson , Maladie de Wolman/anatomopathologieRésumé
One of the greatest challenges of transfusion medicine is the prevention of transmission of infectious diseases through blood transfusion. The aim of this study is to present the current status of transfusion transmitted diseases among healthy unpaid blood donors so as to heighten the awareness of the complications of blood transfusion and make usmore vigilant with regard to the specifics of blood collection and testing as well as judicious use of blood. 8617 donors donated at St. Johns Medical College Hospital Blood bank from 1st. September 1997 to 31st August 1998. The tests done on all units included testing for HIV 1&2, HBsAg, HCV, VDRL and malarial parasites. The seropositivity among donors for HIV was 0.44%, for HBsAg 1.86%, for HCV 1.02% and for VDRL 1.6% No MP Positives were picked up in the study period. The focus has been on HIV so far. We have found the magnitude of hepatitis to be far more than that of HIV. Hence testing for HCV routinely is recommended.