ABSTRACT
Background: The current treatment of haemophilia is replacement of factor VIII or IX which is effective till development of inhibitor against factors. There has been no study on factor VIII inhibitors in Southern Odisha using Nijmegen朆ethesda assay. This study was planned with objectives to screen factor VIII inhibitors in hemophilia-A patients, to do quantitative estimation of it using Nijmegen-Bethesda assay and to explore factors associated with development of inhibitors. Methods: This cross-sectional study was carried out from September 2016 to August 2018 in Department of pathology, MKCG medical college, Berhampur. Haemophilia-A patients coming to MKCG medical college and registered Haemophilia-A cases under Haemophilia society of Berhampur were included. Patients denying consent and having multiple clotting factors deficiencies were excluded. 1.8ml blood was collected. Mixing study was done to screen factor VIII inhibitors and then in positive cases inhibitors level measured by Nijmegen-Bethesda method. All data were analysed using SPSS (version 16.0).Results: 70 cases of Hemophilia-A patients were studied. Majority (50%) were with severe hemophilia-A. 7 patients developed inhibitors where 3 were high and 4 were low responders. Inhibitor level ranged from 0.8 to 64 Nijmegen-Bethesda units. Patients with severe hemophilia A, more than 10 transfusions and who switched to receive recombinant FVIII from other blood products developed inhibitors which were significant.Conclusions: Severity of hemophilia, increase frequency of transfusion and switching of blood products significantly increases chances of inhibitor development and hence intensive inhibitor screening is needed in these cases. Quantification of inhibitor is needed to monitor treatment and to manage bleeding episodes effectively.
ABSTRACT
Introduction: Aplastic anemia is a potentially life-threateningfailure of hematopoiesis, characterized by pancytopenia andhypocellular bone marrow. Aplastic anemia if untreated resultsin very high mortality. Early diagnosis of Aplastic anemias isessential for appropriate management of the patient.Aims and Objectives: The aim of the study was to assess theprevalence of the condition in southern odisha admitted to thisinstitute and to study the clinico-hematological profile for theassessment of severity by using the modified Camitta criteriaaiding in their management protocol.Materials and Methods: This study was carried outprospectively in the Department of Pathology, MKCGMedical College & Hospital during the period of June 2017 toMay 2019. A detailed clinical history, physical examination,Complete Blood Count, CPS, Reticulocyte count, Bone marrowaspiration and Biopsy were performed in each case and theobservations were evaluated using simple and basic statisticaltools.Results: There are 63 diagnosed cases of aplastic anemiaduring the study period. Out of 63 cases 36 cases are male(57.14 %) and 27 cases are female (42.86 %) indicating a malepreponderance of this disease and male to female ratio is 1.3:1. We also found that, Aplastic anemia has a bi modal agedistribution. Fever, generalized weakness and bleeding due tothrombocytopenia are commonest clinical manifestations. All63 cases are sub categorized into 38 Non severe cases (60.32%), 17 severe cases (26.98 %) and 8 cases are very severe(12.70 %).Conclusion: A good knowledge on clinical and hematologicalparameters will certainly aid in early diagnosis of Aplasticanemia and sub-categorization for treatment. But in adeveloping country financial constraints and lack of awarenessforms a major drawback in patient management. So earlydiagnosis of Aplastic Anemia reduce the treatment cost as wellas will decrease mortality.