Iron Overload Under Recognized in Sickle Cell Disease

Undertreated iron overload in patients with sickle cell disease undergoing chronic transfusion therapy may be an underrecognized problem, according to a new study. Nearly one-half of patients with sickle cell disease in the multi-site GRNDaD registry who had iron overload were not on chelation.

“Since GRNDaD sites are academic centers across the country which focus on the management of sickle cell disease, we speculate that the problem of undertreated iron overload nationally is probably both widespread and underrecognized,” Matthew Sears, PhD, of Sidney Kimmel Medical College of Thomas Jefferson University, and colleagues wrote in an abstract at the 60th Annual ASH Meeting and Exposition.

Chronic transfusion therapy is a key part of the prophylactic management of adults and children with high-risk sickle cell disease for management of cerebral vasculopathy, significant end organ damage, or chronic pain. As each transfusion introduces about 250 mg of iron into the blood, monitoring for iron overload is important, and patients found to have overload should be put on iron chelation.

In this study, Sears and colleagues looked at 402 adult patients from the GRNDaD registry, which accrues patients at five urban sickle cell centers. Of the included patients, 255 had phenotypic homozygous disease, 80 had variant sickle cell disease, and 17 had other or unknown genotypes. Iron accumulation was measured using ferritin levels.

The majority of people (94%) with elevated ferritin had phenotypic sickle cell anemia. More than half of patients with sickle cell with a critically elevated ferritin level were on chronic transfusion therapy; 54% of patients with critically high levels were on chelation.

Less than one in four patients had had a liver scan within 3 years (23%) and more than one in three patients with critical ferritin levels and no chelation therapy remained on transfusion therapy, the researchers wrote.

“We anticipate that, as GRNDaD continues to add additional sites, it will evolve as a robust young adult population with sickle cell disease,” the researchers wrote. “GRNDaD may be a model for identifying and addressing deficiencies in current clinical practices for management of sickle cell disease.”

Sears M, et al. Iron overload is under-recognized and under-treated in SCD: a report from the GRNDaD registry. Presented at 60th Annual ASH Meeting and Exposition; December 1-4, 2018; San Diego. Abstract #158.