Seropositivity of hepatitis C, hepatitis B and HIV in chronically transfused beta -thalassaemia major patients

The purpose of this study was to determine the frequency and trend of transfusion transmitted infections [TTI] in chronically transfused beta-thalassaemia major [TM] patients with reference to the duration of transfusions. A cross-sectional study was done on 160 beta-TM patients and 5517 healthy blood donors to find out the prevalence of HCV, HBV and HIV infections. Out of 160 patients, 21 cases [13.1%] were anti-HCV positive, 2 [1.25%] were HBsAg positive. HIV antibodies were not detected in any sample. However, 109 [1.9%] and 104 [1.8%] of 5517 blood donors were positive for HCV and HBV respectively. No donor showed HIV antibodies. Anti-HCV was positive in 9/111[8.4%] thalassaemics [< 10 years of age] while 11/49 [22%] [> 10 years of age] showing significant difference [p = 0.005] among the two groups. For the past 10 - 12 years the screening of blood has reduced the magnitude of the disease significantly as shown by the trend in two age groups. Further improvements need to be done to implement uniform screening throughout the country

JAK2V617F mutation in chronic myeloid leukemia predicts early disease progression

To determine the association of JAK2V617F mutation along with BCR-ABL translocation or Philadelphia chromosome in chronic myeloid leukemia with early disease progression to advanced stages [accelerated phase or blast crisis] and poor outcome. Case series. National Institute of Blood Diseases and Bone Marrow Transplantation, Karachi, from February 2008 to August 2009. All the newly diagnosed cases of BCR-ABL or Philadelphia positive CML were tested for JAK2V617F mutation by Nested PCR. Demographic data, spleen size, hemoglobin levels, white blood cell and platelet counts were recorded. Independent sample t-test was used for age, haemoglobin level and spleen size. Fisher's exact test was applied to compare disease progression in JAK2V617F mutation positive and negative cases. Out of 45 newly diagnosed cases of CML, 40 were in chronic phase, 01 in accelerated phase and 04 in blast crisis. JAK2V617F mutation was detected in 12 [26.7%] patients; 09 [22.5%] in chronic phase, none in accelerated phase and 03 [75%] in blast crisis. During a mean follow-up of 8 months, 03 patients in chronic phase transformed in blast crisis and 02 into accelerated phase. Overall 08 out 0f 11 [73%] JAK2V617F positive patients either had advanced disease or showed disease progression. Only 2 of 20 [10%] available patients, negative for the mutation, showed disease progression by transforming into blast crisis [p < 0.001]. No statistically significant difference was seen in the age, spleen size, haemoglobin levels, white blood cells and platelets counts in JAK2V617F positive patients. JAK2V617F mutation was detected in 26.7% cases of chronic myeloid leukemia. A significant proportion of them showed early disease progression