Abstract
Background: Sickle cell disease (SCD) is caused by a point mutation in the beta globin gene. SCD is characterized by chronic hemolytic anemia, vaso-occlusive events leading to tissue ischemia and progressive organ failure. A chronic inflammatory state is part of the pathophysiology of SCD. Patients with SCD have extremely variable phenotypes, from a mild disease to severe complications including early age death. The spleen is commonly injured in SCD. Early splenic dysfunction and progressive spleen atrophy are common. Splenomegaly and hypersplenism, can also occur with loss of the crucial splenic function. Acute, life-threatening spleen-related complications in SCD are well studied. The implications of the spleen status including asplenia and splenomegaly/ hypersplenism in vaso- occlusive crisis and long-term complications in SCD remain to be determined. Aims: In this study, we compared blood parameters and RBC properties of SCD patients and compared the results between patients with asplenia, to patients with splenomegaly/hypersplenism. In addition, we implemented novel, non-routinely used methods for the evaluation of the functional status of the spleen in those patients. Methods: We evaluated the association between the spleen status with clinical and laboratory parameters in 31 SCD patients divided into two groups: Group a) Patients with asplenia (N=22) (including auto-splenectomy and patients that were splenectomized) vs. Group b) patients with Splenomegaly and or Hypersplenism (N=9). Laboratory studies included: CBC, reticulocyte count, iron metabolism parameters, CRP, Hb variant distribution and D-dimer. Metabolic and morphological red blood cell (RBC) studies included: density gradient (by Percoll), glucose consumption, lactate release and K+ leakage; fetal RBC (F-Cells) and F Reticulocytes, annexin V+, CD71+, oxidative stress measured by GSH presence in RBC and finally HJB count were all analyzed by FC. SEM analysis of RBC was also performed. Results: Patients in group (a) showed significantly higher WBC, platelet, HCT, hemoglobin S, CRP, D-dimer, GGT, cholesterol and transferrin levels than patients in group (b). The percent of hyperchromic RBCs, F-Reticulocytes were lower in patients with asplenia. Important differences were also found between the groups in the studied RBCs metabolic parameters. Summary/Conclusion: Further studies are required to elucidate the effect of the spleen including hyper and hypo-splenia in vaso-occlusive crisis, vascular pathology, and long-term complications of SCD. The benefits and risks of splenectomy compared to chronic transfusion need to be evaluated in clinical trials and the standard approach to manage hypersplenism in SCD patients should be re-evaluated.
Published Version
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