ABSTRACT
To analyze patients suffering from aplastic anemia [AA, peripheral pancytopenia and hypocellular bone marrow in the absence of dysplasia, infiltration and fibrosis] for documenting patient's baseline characteristics and association with various human leucocyte antigens. An observational, cross-sectional study. The National Institute of Blood Disease [NIBD], Karachi, from March 2003 to August 2008. All consecutive patients with confirmed diagnosis of AA were evaluated. Data included the baseline characteristics, complete blood counts [CBC], bone marrow biopsy findings, severity of disease, exposure to drugs or chemicals, viral serology and their HLA expression. The data was analyzed on SPSS programme and frequencies were documented. Among 318 patients, there were 236 [74.21%] males and 82 [25.78%] females. Median age was 16 and 70% belonged to urban population. Drug exposure could be established in 23 [7.23%] of cases, while 4 [1.25%] were HBV surface antigen positive and 7 [2.2%] were HCV antibodies positive. In all, 73 [22.9%] had very severe AA, 195 [61.32%] had severe AA while 50 [15.7%] cases had non-severe AA. HLA B5 [52] showed high expression in 83 patients [26%] in comparison to 5.9% reported in healthy population. AA was found to affect young adult males living in urban areas. HLA B5 [52] showed higher expression in patients with aplastic anemia
ABSTRACT
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
ABSTRACT
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
Subject(s)
Humans , Male , Female , Mutation , Disease Progression , Philadelphia Chromosome , Blast Crisis , Translocation, GeneticABSTRACT
The case report of a 2 year old boy with steroid refractory DBA, treated with allogeneic PBSCT from an HLA matched sibling is presented. Anti-IL2 receptor antibody Daclizumab was used as a prophylaxis for graft versus host disease [GvHD]. Complete recovery without any evidence of GvHD ensued
ABSTRACT
The case report of a 2 year old boy with steroid refractory DBA, treated with allogeneic PBSCT from an HLA matched sibling is presented. Anti-IL2 receptor antibody Daclizumab was used as a prophylaxis for graft versus host disease [GvHD]. Complete recovery without any evidence of GvHD ensued
Subject(s)
Humans , Male , Anemia, Diamond-Blackfan/surgery , Graft vs Host Disease/prevention & control , Stem Cell Transplantation , Transplantation, Homologous , Steroids , Drug Resistance , Receptors, Interleukin-2/immunologyABSTRACT
To present the survival and evaluate the demographic characteristics as risk factors for acute and chronic graft versus host disease [GvHD] in 100 recipients of HLA identical related allogeneic peripheral blood stem cell transplantation. Indications for transplant were non-malignant and malignant haematological disorders. Bu/Cy conditioning was given for haematological malignancies and b-Thalassaemia major, Cyclophosphamide was given in aplastic anaemia. GvHD prophylaxis was Cyclosporin and Methotrexate. The patients received a median nucleated cell dose of 7.93 108/kg. Of 100 recipients, 72 were males and 28 females. Median age was 13.5 years [range 1.5-44]. There were 65 male and 35 female donors. Median age was 15 years [range 4-45]. Grade-I aGvHD was noted in 18 [18%], Grades-II in 6 [6%], Grade-III in 3 [3%] while Grade-IV in 1 [1%] patients. Diagnosis was found to be a significant risk factor for aGvHD. Kaplan Meyer analysis showed that malignancy, aGvHD, recipients above 14 years of age, female patients and engraftment after 12 days were associated with poor outcome. Of 78 patients alive beyond 100 days, 19 [24%] developed cGvHD. Mean follow up was 466 days [range 30-1766]. Median survival of this cohort of patients was 338 days [mean 479 days, 95% CI 72 - 729]. Incidence of acute and chronic GvHD was similar to published data. Grade of aGvHD, extent of cGvHD, female patients and haematological malignancies were associated with higher rate of aGvHD and a worse outcome