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Background: Heart disease represents the main determinant of survival in βthalassemia, but its particular features in the two clinical forms of the disease, thalassemia major (TM) and thalassemia intermedia (TI), are not completely clarified. Methods: We compared clinical and echocardiographic global parameters in 131 TM patients who received regular chelation transfusions and were highly compliant with treatment (mean age, 28 ± 6 years [±SD]), and 74 age-matched, TI patients who did not receive chelation transfusions. Results: Congestive heart failure was encountered in five patients with TM (3.8%; age range, 25 to 29 years) and in two patients with TI (2.7%; age range, 37 to 40 years). Systolic left ventricular (LV) dysfunction (ejection fraction > 55% or shortening fraction < 35%) was only encountered in patients with TM (8.4%). Considerable pulmonary hypertension (systolic tricuspid gradient > 35 mm Hg) was only present in TI (23.0%). In the remaining patients without evident heart disease, cardiac dimensions, LV mass, LV shortening and ejection fractions, and cardiac output were significantly higher in patients with TI. LV afterload was higher in patients with TM. LV diastolic early transmitral diastolic peak flow velocity (E)/late transmitral diastolic peak flow velocity (A) ratio was also higher in TM. Systolic and mean pulmonary artery pressures and total pulmonary resistance were higher in both young and old TI patients. Conclusion: Regular lifelong transfusion and chelation therapy in TM prevented premature heart disease and pulmonary hypertension, although LV dysfunction still occurred and led to heart failure. The absence of regular therapy in TI, in contrast, preserved systolic LV function but allowed pulmonary hypertension development, which also led to heart failure, starting within the fourth decade of life, a decade later compared to TM. [ABSTRACT FROM AUTHOR]

Splenomegaly is a common finding in ß-thalassemia; however, its hemodynamic features and its potential correlations with high output state and hepatic disorders, both also frequent in thalassemia, have not yet been assessed in these patients. Eight ß-thalassemia patients with the indication for splenectomy and no symptoms or signs of heart disease, aged 25.6±5.5 years, were studied. Preoperative assessment included hematological profile, liver biology, hepatitis virus serology, and echocardiography. During splenectomy, splenic artery blood flow and splenic vein pressure were directly measured and liver biopsies were taken. Preoperative echocardiographic data were compared with those of 34 healthy controls. The preoperative cardiac index was significantly elevated in patients (4.8±1.3 vs 3.4±1.1 l/min per m2 in controls,p<0.001). Splenic blood flow, although increased, was not particularly high, being 285±56 ml/min or 0.13±0.04 ml/min per g of splenic mass, representing 4.1±0.9% of total cardiac output (CO). Splenic vein pressure was considerably elevated (29.7±5.5 cmH2O). Hepatic fibrosis, iron deposition, and extramedullary foci were found in all eight biopsies. Serology was positive in five of eight cases. ß-thalassemia patients with extensive splenomegaly requiring splenectomy are characterized by high output state, increased splenic blood flow, which probably makes a limited contribution to CO elevation, and portal hypertension, manifest by increased splenic vein pressure and hepatic histopathological abnormalities. [ABSTRACT FROM AUTHOR]

We describe a 27-year-old male patient suffering from β-thalassemia intermedia who presented with a nontraumatic spontaneous hemothorax due to extramedullary hemopoietic foci. In reviewing the literature, four similar reports were found. The details of this unusual entity are discussed. [Copyright &y& Elsevier]

A case of pyomyositis is presented. This case is unique in the literature as at least 29 abscesses were detected, affecting the vast majority of big muscle groups. We outline the origin of this diseaseentity which selectively affects striated muscles. We also discuss its natural history and management strategy. [ABSTRACT FROM AUTHOR]