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
Background: Sickle Cell Disorder (SCD) is a major health problem in India. After introduction of High-Performance Liquid Chromatography (HPLC) in MKCG Medical College, this study is first of its kind to describe haemoglobin variants of SCD. The aim of the study was to document haematological profile and pattern of haemoglobin variants in SCD patients.Methods: A Hospital based cross sectional study was conducted in Pathology department, MKCG medical college from October 2018 to May 2019. Sickle cell patients were included and patients in Sickle cell crisis or transfused with blood in last 3 months were excluded. Hematological indices were measured by Sysmex XT 2000i blood analyzer. Quantification of hemoglobin variants was done by HPLC. All data were analyzed using SPSS and Independent t-test was applied.Results: In this study 100 heterozygous and 116 homozygous cases were reported. In homozygous cases Hb were significantly low and MCV, MCH, RDW-CV were significantly high than heterozygous. Hb level was significantly lower in homozygous children. Hb F was significantly higher in children and homozygous cases. A significant positive correlation was seen between Hb and RBC in both cases.Conclusions: In homozygous cases moderate anaemia (microcytic hypochromic to normocytic hypochromic) with High Hb F and in heterozygous cases mild anaemia (microcytic hypochromic) dominated the haematological profile. Children were significantly more anaemic than adults in homozygous cases. Anisocytosis was significantly more in homozygous cases and pediatric age group. Average fetal Haemoglobin variant (Hb F) was significantly more in homozygous cases and lower in adult group in both homo and heterozygous cases.