We describe outcomes after human leukocyte antigen-matched sibling bone marrow transplantation (BMT) for 179 patients with ␤-thalassemia major. The median age at transplantation was 7 years and the median follow-up was 6 years. The distribution of Pesaro risk class I, II, and III categories was 2%, 42%, and 36%, respectively. The day 30 cumulative incidence of neutrophil recovery and day 100 platelet recovery were 90% and 86%, respectively. Seventeen patients had graft failure, which was fatal in 11. Six of 9 patients with graft failure are alive after a second transplantation. The day 100 probability of acute graft-versus-host disease and 5-year probability of chronic graftversus-host disease was 38% and 13%, respectively. The 5-year probabilities of overall-and disease-free survival were 91% and 88%, respectively, for patients with Pesaro risk class II, and 64% and 62%, respectively, for Pesaro risk class III. In multivariate analysis, mortality risks were higher in patients 7 years of age and older and those with hepatomegaly before BMT. The leading causes of death were interstitial pneumonitis (n ‫؍‬ 7), hemorrhage (n ‫؍‬ 8), and veno-occlusive disease (n ‫؍‬ 6). Proceeding to BMT in children younger than 7 years before development of end-organ damage, particularly in the liver, should improve results after BMT for ␤-thalassemia major. (Blood. 2011;117(5):1745-1750)

BACKGROUND:Limited information is available from developing countries in patients of non-Hodgkin's lymphoma (NHL). Hence, we reviewed the demographical profile along with bone marrow infiltration pattern in patients with NHL presented at Liaquat National Hospital and Medical College.MATERIALS AND METHODS:In this descriptive study, adult patients with NHL were enrolled from January 2011 to December 2015.RESULTS:One hundred and Eighty-four histopathologically confirmed cases of NHL were identified. There were 139 males and 45 females, with a male-to-female ratio of 3:1. The mean age was 48.5 ± 16.0 years with the median age of 50 years. B-symptoms were present in 80.4% of patients. Lymph node enlargement was present in 71.1% of the cases. One hundred and sixty-eight patients had B-cell lymphoma (91.3%) and 16 patients had T-cell lymphoma (8.6%). Overall 158 (85.8%) patients had aggressive lymphoma. The frequency of bone marrow infiltration in our NHL patients was found to be 31.5%. Pattern of infiltrate was diffuse (14.6%) predominantly followed by interstitial (6.5%) and paratrabecular (5.4%) types of infiltration. The least common was nodular infiltrate accounted in 9 (4.8%) patients.CONCLUSIONS:B-cell lymphoma is more frequent than T-cell lymphoma. Younger age, aggressive lymphoma, and predominance of B-symptoms are more frequently seen. Diffuse bone marrow infiltration is more common in our set up probably because of a relatively late presentation in our patients.

PurposeThe primary study purpose is to examine whether managerial support triggers job crafting and sustainable employability, and to what extent work uncertainty moderates the managerial support and sustainable employability relationships mediated by job crafting using a moderated mediation approach. Thus, this study aims to uncover new antecedent and outcomes of job crafting using job demands and resources (JD–R) theory as no such research has yet examined these relationships.Design/methodology/approachThe structural equation modeling (SEM) technique was employed to test the proposed relationships based on survey data that include a final 483 knowledge workers of the services sector. The authors tested the structural model using self-developed estimates for AMOS 24.0 to examine the moderated mediation process models (process models 7, 14 and 58) rather than using a conventional process macro through SPSS.FindingsConsistent with the formulated hypothesis, the results of this study indicate that managerial support directly stimulates job crafting and sustainable employability. Further, job crafting mediates the relationships between managerial support and sustainable employability. This validates the JD–R theory assumption that managerial support as job resources initiates a motivational process through job crafting, leading to sustainable employability as the outcome of the motivational process. Additionally, the moderated mediation results show that in the presence of high work uncertainty, employees are more engaged in job crafting to boost their sustainable employability.Practical implicationsOrganizations may incorporate these findings while developing human resources (HR) management policies and practices to align top-down and bottom-up job re(design) approaches. For example, by designing line managers’ role in implementation of supportive HR practices, their supportive leadership behavior towards employees will successfully trigger job crafting and nurture their sustainable employability.Originality/valueThis research adds to the work design and employability literature. No such study has yet examined whether managerial support triggers job crafting and sustainable employability. Prior studies examine personality traits, some individual difference variable, job characteristics, or leadership influence as antecedents of job crafting. Utilizing the JD–R theory, we empirically validate that job crafting plays a vital role in the motivational process initiated by the job resources (managerial support), leading to sustainable employability as an outcome of the motivational process. The authors further show that in the presence of high job demands (work uncertainty), employees are more engaged in job crafting and more conscious to boost their sustainable employability.

Objective: To determine the prevalence of Hepatitis-B, Hepatitis-C and Human Immunodeficiency infections in replacement blood donors.Materials and Methods: From January 2004 to December 2011, 108,598 apparently healthy donors donated blood at our Blood Bank. Screening was done by Microparticle Enzyme Immuno Assay (MEIA) method on Axsym System (Abbott Diagnostic, USA) and in year 2011 by Chemiluminescent Immunoassay (CIA) method on Architect i2000 (Abbott Diagnostic, USA). From 2010 onward, HIV reactive donors were advised for confirmatory tests and reported back with the results.Results: Of the 108,598 total donors, 108,393 (99.8%) were replacement donors with a mean age of 28.92 (17-55) years. Of this, only 164 (0.15%) were females. Among the replacement donors, 4,906 (4.5%) were found to be reactive for Hepatitis-B, C and Human Immunodeficiency Virus. All the reactive patients, except one, were males. HbsAg was positive in 2,068 (1.90%) and anti-HCV in 2832 (2.61%) donors, while 111 (0.10%) were positive for Human Immunodeficiency Virus. Co-infectivity was observed in 103 (0.09%) cases. The prevalence appeared to be higher in younger age group (17-30 yrs). Only 16.6% cases should be patients returned with results of the confirmatory tests for HIV and were found positive.Conclusion: Hepatitis-B and C sero-prevalence in our series of replacement donors appears high compared to most studies from neighboring countries and relatively low in comparison to earlier studies from Pakistan. Prevalence of HIV, however, appears low and turn out of HIV positive cases for confirmatory tests is low.Conflict of interest:None declared.

Background: Primary myelodysplastic syndrome (MDS) is an acquired clonal disorder of myeloid progenitor cells, characterized by peripheral cytopenias in the presence of hypercellular marrow with dysplastic features. Our aim was to study the demographical and clinicopathological features of adult Pakistani patients with MDS at disease presentation. Materials and Methods: This single centre study was conducted at Liaquat National Hospital and Medical College, extending from January 2010 to December 2014. Data were retrieved from the patient archives. Results: Overall 45 patients were diagnosed at our institution with de novo MDS during the study period. There were 28 males and 17 females. Age ranged between 18 and 95 years with a mean age of 57.6±17.4 years and median of 64 years. The male to female ratio was 1.7:1. The main presenting complaints were generalized fatigue (60%), fever (33.3%), dyspnea (15.5%), bleeding (13.3%) and weight loss (11.1%). Examination was unremarkable in 42.2% of patients. Physical examination revealed pallor in 37.7%, followed by petechial and purpuric rashes in 20%. The commonest laboratory finding was anemia (hemoglobin < 10 g/dl in 41 (91.1%) patients. Out of these, 27 (60%) patients had normocytic anemia, followed by macrocytic (22.2%) and microcytic (8.8%). Conclusions: Primary MDS in Pakistani patients demonstrates a male preponderance. The proportion of anemic patients was high in our series with predominance of normocytic anemia. However, other clinico-hematological features appear comparable to published data.