Cardiac disease in beta-thalassaemia major: Is it reversible?

INTRODUCTION: The aim of this study was to evaluate the spectrum of cardiac involvement and its outcome in beta-thalassaemia major. METHODS: There were 75 patients with a mean age of 13.8 (+/- 5.5) years, of whom 33 were male and 42 were female. Clinical history, examination and laboratory investigations were assessed. Electrocardiograms, chest radiographs and echocardiograms were reviewed. RESULTS: 44 patients had cardiac involvement in the form of left ventricular systolic dysfunction in 17, diastolic dysfunction in 22, pericardial effusion in 12 and pulmonary hypertension in 12 patients. With intense chelation therapy and cardiac medications, the condition of 13 of 17 patients with systolic dysfunction, and four of 22 with diastolic dysfunction, improved. CONCLUSION: Cardiac disease is a common complication of siderotic disease in thalassaemia major and it can be prevented with regular chelation. This study has shown improved systolic function after regular chelation therapy.

Use of intravenous anti-D in patients with refractory and relapsed immune thrombocytopenic purpura.

OBJECTIVE: To determine the response to i.v. anti-D and its comparison with splenectomy as second line therapy in refractory and relapsed cases of ITP, in the Aga Khan University Hospital, Karachi. METHODS: A total of 23 patients with chronic ITP were treated with either anti-D or splenectomy as second line treatment. The patients were assessed for time to achieve a response to second line treatment, duration of response and adverse events. RESULTS: There were 12 patients in the anti-D group and 11 in the splenectomy group. The mean platelet count at presentation was 9,000/cumm. The mean age was 8.9 years and 13.0 years and the male to female ratio was 1:1 and 1:1.2 in anti-D and splenectomy group respectively. 54.5% of the patient in the anti-D group responded compared to 81.8% in the splenectomy group. Median time to achieve a response was 7 days in the anti-D group and 1 day in the splenectomy group. Mean time to relapse was 87.8 days in the anti-D group and 55.4 days in the splenectomy group. No adverse events were recorded for any of the infusions of anti-D and none of the patients had more than 0.5 gm/dl fall in the hemoglobin level following anti-D infusion. CONCLUSION: It was thus concluded that Anti-D is a relatively safe, convenient and effective therapy for chronic ITP and can be used as a splenectomy sparing agent when treatment is clinically indicated.