Morbidity and mortality of sickle cell disease patients is unaffected by splenectomy: evidence from three decades of follow-up in a high-income setting

Abstract

Not available.

Increased risk of venous thromboembolism in splenectomized patients with sickle cell disease

“…Few reports have compared the SS and Sβ 0 phenotypes, but Sβ 0 patients might be more prone to persistent splenomegaly 6,7 and ASS 8 ; characteristics that may result in a higher rate of splenectomy, as observed as early as 1979 9 and in a recent Italian cohort (n = 534, 187 SS and 176 Sβ 0 ) showing that 53% of Sβ 0 patients had a history of splenectomy versus 9.6% of SS patients. 2 Coexistent α-thalassaemia may also cause persistent splenomegaly. 10…”

“…1 In addition, unpredictable complications, such as acute splenic sequestration (ASS) and hypersplenism, may require surgical splenectomy in up to 30% of patients. 2 Some authors have reported an increase in acute chest syndrome (ACS) and vaso-occlusive crisis (VOC) in the few years following the procedure in children, 3 but data on the long-term consequences of splenectomy in SCD adults are scarce. In conditions other than SCD, increases in venous thromboembolic events (VTEs) and pulmonary hypertension (PHT) have been reported following splenectomy.…”

Splenectomy in sickle cell disease: do benefits outweigh risks?

“…In a letter to the editor published in this issue of Haematologica, Pinto et al (10) compared survival and long-term complications in splenectomised and non-splenectomised individuals with SCD in Italy.…”

“…There is a general paucity of research on the spleen in SCD. Although the study by Pinto et al (10) provides much needed novel evidence on the absence of long-term mortality related to surgical splenectomy, data on potential benefits of splenectomy are still sparse. Overall, international guidelines on the management of ASS and hypersplenism are lacking and recommendations for surgical splenectomy are based on weak evidence and pertain to all types of SCD despite genotypic variations in age and clinical presentation (8,9).…”

“…Additionally, most children with SCD become functionally asplenic at a very young age, adding to the complexity of a surgical risk evaluation as outcomes on morbidity and mortality related to the spleen are very difficult to interpret. In a Letter to the Editor published in this issue of Haematologica , Pinto et al 10 compared survival and longterm complications in splenectomized and non-splenectomized individuals with SCD in Italy. Presenting retrospective data from 534 individuals with SCD, 117 (32%) of whom had undergone surgical splenectomy, the study provides novel evidence on the absence of longterm increased mortality related to surgical splenectomy in SCD.…”