A chronic hypercoagulable state and life-long platelet activation in beta thalassemia major.

Increased frequency of thromboembolic events has been recently observed in patients with thalassemia major (TM), causing hypoxemia and cor pulmonale. Autopsy findings demonstrated "old" and recent pulmonary and renal infarcts as well as premature atherosclerosis. Studies to determine hypercoagulability showed: impaired platelet aggregation, increased circulating platelet aggregates, shortened platelet survival, enhanced excretion of urinary metabolites of thromboxane A2 (TXA2) and prostacyclin and decreased plasma levels of Protein C, Protein S or anti-thrombin III. Erythrocytes from TM patients enhanced thrombin formation in a "prothrombinase" assay (using a chromogenic substrate). Chronic anti-thrombotic therapy may be indicated in thalassemic patients to prevent the cardiac and pulmonary complications.

The response to treatment in chronic idiopathic thrombocytopenic purpura (ITP).

Immune thrombocytopenic purpura (ITP) is a relatively common illness. The initial recommended therapy is glucocorticoids, however due to low response rate and high rate of relapses, the second preferred line of therapy is splenectomy. About 60% of the patients respond to this therapy. A retrospective study performed in Israel in 226 children and 446 adults with ITP has evaluated the response to steroids and splenectomy. The response rates were similar to reported studies and no significant differences were observed between children and adults. The possibilities of treating patients who fail splenectomy and exhibit refractory disease are summarized.