Our aim was to study the cause and describe the clinical features of pulmonary arterial hypertension (PHT) in splenectomized beta-thalassemia (beta-Thal) patients. Ten splenectomized beta-Thal patients with systolic pulmonary artery (PA) pressure >30 mm Hg were evaluated by echocardiography, right-heart catheterization, and pulmonary angiography. Five of these patients later underwent hemodynamic studies. Echocardiography and pulmonary angiography on the 10 patients showed normal values of left ventricular systolic function and no findings of acute or chronic pulmonary embolism. Hemodynamic evaluation showed very high PA pressures associated with markedly increased pulmonary vascular resistance indices (PVRIs). Hematological evaluation of the 10 patients showed marked anemia, markedly increased numbers of nucleated red blood cells (nRBCs), and serum ferritin. Mean platelet count, plasma beta2 thromboglobulin, and thrombin-antithrombin III complex levels were significantly increased. It was concluded that PHT can be found in splenectomized beta-Thal patients. Features associated with PHT were female sex, hemoglobin E/beta-Thal, status many years postsplenectomy, marked anemia, markedly increased nRBC count, thrombocytosis, and very high serum ferritin levels. PHT was not due to pulmonary emboli. Our findings suggested that severe PHT was due to increased PVRI from thrombotic pulmonary arteriopathy, likely from chronic low-grade hypercoagulability and platelet activation after splenectomy.
Summary:Here, we report a case of paediatric -thalassaemia Patients with autoimmune haemolytic anaemia (AIHA) after allogeneic transplantation are usually treated with either steroids alone or multiple immunosuppressive therapies, including antithymocyte globulin, cyclophosphamide, immunoglobulin, splenectomy, vincristine and erythropoietin. The overall response to these therapies has been generally unsatisfactory.1 Quartier et al 2 described the treatment of childhood refractory AIHA with rituximab. Therefore, we would like to report our experience about a successful treatment of refractory AIHA in a post-unrelated allogeneic bone marrow transplant pediatric patient with rituximab.
Rituximab therapy for refractory AIHA post unrelated BMTA 37-month-old Thai boy who had -thalaessaemia major underwent unrelated T cell-nondepleted bone marrow transplantation. His course of transplant was uneventful with full donor engraftment. On day 180 post transplant, the child
Recombinant activated factor VII (rFVIIa), combined with local measures of fibrin glue and a celluloid splint, preventing bleeding from four invasive dental procedures is reported. A single dose of 180-200 micro g/kg was successfully used in three surgical removals of impacted teeth. Four doses of rFVIIa were required in another full mouth treatment of extraction, pulpotomy, filling and the stainless steel crowning of 13 teeth. The repeated dose of rFVIIa was given whenever the bleeding complication was visualized. It is cost-effective for preventing external bleeding. Additionally, an oral rinsing solution of tranexamic acid (25 mg/kg) was given three times a day for 7 days. In conclusion, rFVIIa has been shown to be an effective alternative to platelet concentrate in patients with Glanzmann thrombasthenia.
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