The following article features coverage from the American Society of Hematology 2021 meeting. Click here to read more of Hematology Advisor‘s conference coverage.

Adult patients with sickle cell disease (SCD) showed lower cognitive scores in a variety of domains compared with healthy control individuals, with differences based on chronic blood transfusion history. These findings were presented at the 2021 American Society of Hematology (ASH) Annual Meeting by Gerard Portela, MPH, of the University of Pittsburgh in Pennsylvania, and colleagues.

Researchers compared cognitive function in patients with SCD with that of healthy control individuals based on factors like stroke history and transfusion history. Patients with SCD were recruited through the University of Pittsburgh Medical Center Adult Sickle Cell Program. Healthy control individuals were matched by sex and age for comparisons.

Cognitive status was evaluated through multiple types of assessments. Linear regression was used to analyze cognitive scores for patients with SCD versus control individuals. Multivariate analyses were performed to evaluate scores in cognitive domains based on stroke and transfusion history in comparisons.

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The study included 86 patients with SCD and 66 control individuals. Among patients with SCD, 14.0% had a stroke history and 19.8% were recipients of chronic transfusions. None of the individuals in the control arm had a stroke or transfusion history.

Linear regression suggested that patients with SCD generally showed worse scores than healthy control individuals in several cognitive domains. In an adjusted analysis, these included attention (P =.002), Repeatable Battery for the Assessment of Neurophysiological Status (RBANS) for processing speed total score (P =.006), and the Digit Symbol Substitution Test (DSST) scaled score (P=.022).

Multivariable adjusted analyses revealed worse executive function, RBANS total, and DSST scores for patients with SCD and a stroke history compared with those with SCD and no stroke history. Persons with SCD and a stroke history also had lower attention, executive function, RBANS total, and DSST scores compared with control individuals. Persons with SCD and no stroke history also showed worse attention and RBANS total scores than control individuals did.

Within the SCD population, multivariable adjusted analyses did not reveal differences in cognitive function based on presence or absence of transfusions. Additionally, patients with SCD receiving chronic transfusions did not perform differently compared with individuals in the healthy control arm.

Patients with SCD and no transfusions performed significantly worse in some domains compared with control individuals, however. “Most notably, in our adjusted linear model, we found that sickle cell patients who were not receiving transfusions had significantly lower attention, RBANS total, and DSST scaled scores,” Portela said during a presentation accompanying the research team’s poster.

The researchers concluded that adjusted analyses indicated patients with SCD showed significant impairments in attention, global cognitive function, and psychomotor speed compared with control individuals. Portela also noted that the presence of some cognitive deficits in patients with SCD who did not have a stroke history suggests an additional aspect of SCD contributes to cognitive impairment.

Disclosure: One study author declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.

Read more of Hematology Advisor‘s coverage of the ASH 2021 meeting by visiting the conference page.


Portela G, Butters M, Brooks MM, Candra L, Rosano C, Novelli AM. Comprehensive assessment of cognitive function in patients with sickle cell disease reveals deficits in memory and processing speed. Presented at ASH 2021; December 11-14, 2021. Abstract 975.