To cite this article: Rodrigo C, Gooneratne L. Dapsone for primary immune thrombocytopenia in adults and children: an evidence-based review. J Thromb Haemost 2013; 11: 1946-53. Summary. Primary immune thrombocytopenia is a potentially life-threatening condition. Approximately two-thirds of adult patients do not have a sustained response to steroids (first-line therapy). For these patients, a number of other treatment options exist, such as rituximab, splenectomy, immunosuppressants, and thrombopoietin receptor agonists, but they are costly and have side effects. Dapsone is an inexpensive drug with a well-established safety profile. Unfortunately, this treatment option has not been explored adequately. This review is aimed at analyzing the currently available evidence for the use of dapsone as second-line or third-line therapy in primary immune thrombocytopenia.

Between 2005 and 2015, 138,165 hematopoietic stem cell transplantation (HSCT) were reported in 18 countries/regions in the Asia-Pacific region. In this report, we describe current trends in HSCT throughout the Asia-Pacific region and differences among nations in this region and various global registries. Since 2008, more than 10,000 HSCTs have been recorded each year by the Asia-Pacific Blood and Marrow Transplantation Group Data Center. Between 2005 and 2015, the greatest increase in the number of HSCTs was observed in Vietnam. Allogeneic HSCT was performed more frequently than autologous HSCT, and a majority of cases involved related donors. Regarding allogeneic HSCT, the use of cord blood has remained steady, especially in Japan, and the number of cases involving related HLA non-identical donors has increased rapidly, particularly in China. The incidence of hemoglobinopathy, a main indication for allogeneic HSCT in India, China, Iran, and Pakistan, increased nearly six-fold over the last decade. Among the 18 participating countries/regions, the transplant rate per population varied widely according to the absolute number of HSCTs and the national/regional population size. We believe that this report will not only benefit the AP region but will also provide information about HSCT to other regions worldwide.

BackgroundEarly detection and treatment of iron deficiency during pregnancy is crucial for optimum pregnancy outcomes. Anaemia is a late indictor of iron deficiency measured as Hb < 11 g/dL, and is widely used as a proxy for iron deficiency. We aimed to evaluate the role of red cell indices as a screening tool for early detection of iron deficiency among pregnant women in an urban area of Sri Lanka.MethodA cross-sectional study was conducted among 110 apparently healthy pregnant women ≤12 weeks of gestation attending antenatal clinics in Colombo, Sri Lanka. Women already on nutritional supplements were excluded. Full blood count, serum ferritin (SF) and high sensitive C-reactive protein (hs-CRP) assessments were performed. The women with evidence of inflammation as indicated by hs-CRP > 10 mg/L were excluded (N = 20) from data analysis. Anaemia (Hb < 11 g/dL) and iron deficiency (SF < 30 μg/L) were defined according to WHO guidelines. Receiver operating characteristics curves were used to derive red blood cell indices that showed the optimal cut-offs in detecting early iron deficiency.ResultsOf the 90 women, 63 (70.0%) were iron deficient (SF < 30 μg/L), out of whom 10 (15.9%) were identified as having iron deficiency anaemia (Hb < 11 g/dL). A high sensitivity (> 70%) in the prediction of iron deficiency was obtained for the optimal cut-off values of Hb < 12.2 g/dL, MCV < 83.2 fl, MCH < 26.9 pg and MCHC 33.2 g/dL while maintaining a specificity > 40%.ConclusionIron deficiency can be predicted in early stages using Hb and red cell indices, which is much less expensive. This could be a useful method in areas with limited resources and a high prevalence of iron deficiency.

Hump-nosed viper bites are frequent in southern India and Sri Lanka. However, the published literature on this snakebite is limited and its venom composition is not well characterized. In this case, we report a patient with thrombotic thrombocytopenic purpura-like syndrome following envenoming which, to the best of our knowledge, has not been reported in the literature before. A 55-year-old woman from southern Sri Lanka presented to the local hospital 12 hours after a hump-nosed viper (Hypnale hypnale) bite. Five days later, she developed a syndrome that was characteristic of thrombotic thrombocytopenic purpura with fever, thrombocytopenia, microangiopathic hemolysis, renal impairment and neurological dysfunction in the form of confusion and coma. Her clinical syndrome and relevant laboratory parameters improved after she was treated with therapeutic plasma exchange. We compared our observations on this patient with the current literature and concluded that thrombotic thrombocytopenic purpura is a theoretically plausible yet unreported manifestation of hump-nosed viper bite up to this moment. This study also provides an important message for clinicians to look out for this complication in hump-nosed viper bites since timely treatment can be lifesaving.

Key Points• In low-risk BMT for thalassemia, ATGBuCy seems as effective as Tt-BuCy in the prevention of rejection and may decrease transplant-related mortality. received ATG-BuCy. All patients were ,15 years and had no hepatomegaly (liver #2 cm from costal margin). Actuarial overall survival in the Tt-BuCy and ATG-BuCy groups was 87% and 94% and thalassemia-free survival was 80% and 85% at a median follow-up of 37 and 17 months, respectively, with no significant differences by log-rank statistics. SubstitutingTt with ATG in the standard BuCy context seems safe and effective and may decrease transplant-related mortality. Higher fertility rates are expected for patients who received ATG-BuCy.