Markers of ineffective erythropoiesis in non-transfusion dependent β-thalassaemia

@#Non-transfused β-thalassaemia patients develop complications related to unsuppressed ineffective erythropoiesis (IE). Serum markers of IE would be useful for risk stratification and monitoring treatment. We studied βthalassaemia trait (β-TT) and non-transfusion-dependent βthalassaemia (β-NTDT) patients. Serum erythropoietin (EPO) and soluble transferrin receptor (sTfR) were correlated against markers of clinical severity (haemoglobin, LDH, retics, bilirubin, spleen size) and iron overload (ferritin, hepcidin, and MRI-T2* in NTDT patients). Eleven β-NTDT and nine β-TT subjects were studied. βNTDT patients had significantly higher markers of haemolysis and iron overload. In β-NTDT, liver iron ranged from mild to severe, but no cardiac loading was seen. EPO and sTfR were higher in patients with β-NTDT than β-TT, and correlated significantly with each other (ρ=0.630, p=0.003). Both markers were negatively correlated with haemoglobin (sTfR ρ=-0.540, p=0.014; EPO ρ=-0.807, p<0.001, and positively correlated with spleen size (sTfR ρ=0.783, p<0.001; EPO ρ=0.654, p=0.002) and markers of iron overload. There was a strong correlation between ferritin and hepcidin (ρ=0.720, p<0.001), and a relatively lower increment of hepcidin for the degree of iron overload in βNTDT compared to β-TT. EPO and sTfR appear to be reliable markers of erythropoiesis in non-transfused β-thalassaemia and correlate well with markers of disease severity. Their role in managing patients, predicting complications, and monitoring response to treatments aimed at reducing IE should be explored.

Severe cutaneous adverse drug reactions: Stevens Johnson Syndrome and toxic epidermal necrolysisa, a report of 4 cases seen at UMMC

Prescribing medication is not without its adverse effects. Complications due to drug therapy are on the rise in Malaysia, especially when antibiotics are used indiscriminately. We reviewed cases admitted to the Acute Medical Ward of University of Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia, over a two-month period from March to April 2009. The authors found that Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) were the most common severe adverse cutaneous reactions due to ingestion or parenteral use of drugs. In this report, is a brief description of the two conditions and ways to manage them. The authors have come to a conclusion that judicious use of medications with adequate patient education is important in order to avoid these adverse effects