The increased use of transfusions may ultimately be balanced by hydroxyurea and other newer therapies developed as the complex pathophysiology of SCD is better understood; however, red cell transfusion is currently the most studied and accepted therapy for most acute and many chronic complications of SCD. Physicians caring for patients with sickle cell disease should be aware of the unique complications and transfusion requirements in this population.

Chronic ECP should be considered in patients requiring optimal management of HbS levels and iron burden. Concerns about increased alloimmunization with ECP may be unjustified.

The Friday afternoon admission of a child with a potential diagnosis of leukemia creates perceived delays in treatment initiation. Although generally not felt to affect prognosis, the effect of a few days delay in chemotherapy for children with acute lymphoblastic leukemia (ALL) has not been fully investigated. We retrospectively analyzed 207 patients consecutively diagnosed with ALL at Children's Hospital & Research Center Oakland from 1995 to 2007 to determine if delay in chemotherapy increased the risk of relapse, death, transfer to the intensive care unit, or bacteremia. Friday admission did not significantly delay chemotherapy initiation with treatment started at a mean of 4.13±2.40 days for Friday admits versus 3.72±1.57 days for all others (P=0.29). There was no significant association between treatment delay days and relapse (P=0.94) or death (P=0.55). In Cox regression analysis, treatment delay was not a predictor of time to relapse (P=0.80) or longer duration of hospitalization (corrected for delay, P=0.15). There were trends toward significant associations between treatment delay and bacteremia (P=0.07) and intensive care unit admissions (P=0.08), although both were associated with shorter, not longer, treatment delays.We were unable to demonstrate a significant effect of delay in chemotherapy initiation for pediatric patients with newly diagnosed ALL on the examined outcome variables.

Pulmonary hypertension (PH) has been reported with nearly all forms of the inherited as well as the acquired hemolytic anemias. Recent research investigating the pathophysiology of PH in sickle cell disease and thalassemia has helped elucidate the central role of hemolysis-mediated endothelial dysfunction in the development of PH in these populations. Although the most appropriate treatment of PH in patients with hemolytic anemia is not clearly defined, the associated significant increased risk of death underscores the need for randomized clinical trials in this area.