Adult patients with sickle cell disease (SCD) who undergo a splenectomy are at increased risk for venous thromboembolic events (VTEs), according to findings from an observational, retrospective study published in the British Journal of Haematology.

About 30% of individuals with SCD may require a surgical splenectomy because of such unpredictable complications as acute splenic sequestration and hypersplenism. Recognizing that among persons with disorders other than SCD, increased rates of VTEs have been observed after splenectomy, the researchers sought to compare the features of splenectomized adults with SCD with those of nonsplenectomized adults with SCD. VTEs included deep vein thrombosis (DVT) and pulmonary embolism (PE).

The current analysis was conducted at the adult SCD Referral Centre of the European Georges Pompidou Hospital in Paris, France. All patients with SCD who had received regular care at the institution between October 2015 and May 2021 were included in the study.

Data regarding ongoing and prior therapies, demographics, baseline biological parameters, and occurrence of acute and chronic SCD complications (eg, VTEs, pulmonary hypertension, acute chest syndrome [ACS], and vaso-occlusive crisis) was compared between the splenectomized and nonsplenectomized SCD groups.

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Three hundred sixty patients with SCD were included in the study. Among them, 11.7% (42 of 360) had received a splenectomy at a median age of 9.5 years (range, 7.2 to 15.5 years). The median duration of postsplenectomy follow-up was reported to be 12.9 years (range, 9.5 to 17.4 years).

Results of the study showed that overall, the splenectomized vs nonsplenectomized participants were significantly younger (median age, 23.2 vs 29.2 years, respectively; P =.006) and were diagnosed significantly more often with the Sβ0 genotype of SCD (21.4% vs 2.2%, respectively; P <.001).

Following adjustment for age and SCD genotype (ie, SS genotype or Sβ0 genotype), presence of the α-thalassemic trait was shown to be significantly associated with an individual having undergone a splenectomy (odds ratio [OR], 3.3; 95% CI, 1.3-11.3; P =.015). In addition, among splenectomized vs nonsplenectomized participants, the use of hydroxyurea was significantly more common (90.2% vs 74.5%, respectively; P =.042) and was started at a significantly younger age (15.8 vs 21.2 years, respectively; P =.001).

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The median patient age at first VTE was 27.5 years (range, 21.8 to 31.4 years). Further, the median time to initial VTE following splenectomy was 12.3 years (range, 2.2 to 21.6 years). Splenectomized vs nonsplenectomized participants had a higher prevalence of VTE (25% vs 12.5%, respectively; P =.059).

Those who had undergone splenectomy had a 3.8-fold increased risk for developing non-ACS-related DVT or PE compared with those who had not (95% CI, 1.2-11.2; P =.015). Risk for non-ACS-related PEs was independently linked to receiving a splenectomy (OR, 4.55; 95% CI, 1.1-16.6; P =.025).

The authors emphasized that, “[A]lthough the spleen is often overlooked in SCD because its function is supposed to be lost early in the course of the disease, preserving splenic tissue might be more important than previously thought.”


Tennenbaum J, Volle G, Pouchot J, et al. Increased risk of venous thromboembolism in splenectomized patients with sickle cell disease. Br J Haematol. Published online March 14, 2023. doi:10.1111/bjh.18743