Partial splenic embolization in children with hereditary spherocytosis

Pratl, B; Benesch, Martin; Lackner, Herwig; Portugaller, Horst; Pusswald, Bernadette; Sovinz, Petra; Schwinger, Wolfgang; Moser, Andrea; Urban, Christian

doi:10.1111/j.1600-0609.2007.00979.x

Cited by 17 publications

(12 citation statements)

References 24 publications

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“…The ability to distinguish splenectomized patients from non-splenectomized patients can be valuable in an era where different types of splenectomy are explored. Partial splenectomy, either through embolization of splenic arteries or through (laparoscopic) removal of a part of the spleen, might ameliorate symptoms and improve anemia while maintaining splenic phagocytic function (Tchernia et al, 1993;Pratl et al, 2008). However, data of several small studies are inconclusive regarding the remaining immunological capacity of the spleen after partial splenectomy even though hemolysis is decreased (Guizzetti, 2016).…”

Section: Discussionmentioning

confidence: 99%

Ektacytometry Analysis of Post-splenectomy Red Blood Cell Properties Identifies Cell Membrane Stability Test as a Novel Biomarker of Membrane Health in Hereditary Spherocytosis

et al. 2021

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Hereditary spherocytosis (HS) is the most common form of hereditary chronic hemolytic anemia. It is caused by mutations in red blood cell (RBC) membrane and cytoskeletal proteins, which compromise membrane integrity, leading to vesiculation. Eventually, this leads to entrapment of poorly deformable spherocytes in the spleen. Splenectomy is a procedure often performed in HS. The clinical benefit results from removing the primary site of destruction, thereby improving RBC survival. But whether changes in RBC properties contribute to the clinical benefit of splenectomy is unknown. In this study we used ektacytometry to investigate the longitudinal effects of splenectomy on RBC properties in five well-characterized HS patients at four different time points and in a case-control cohort of 26 HS patients. Osmotic gradient ektacytometry showed that splenectomy resulted in improved intracellular viscosity (hydration state) whereas total surface area and surface-to-volume ratio remained essentially unchanged. The cell membrane stability test (CMST), which assesses the in vitro response to shear stress, showed that after splenectomy, HS RBCs had partly regained the ability to shed membrane, a property of healthy RBCs, which was confirmed in the case-control cohort. In particular the CMST holds promise as a novel biomarker in HS that reflects RBC membrane health and may be used to asses treatment response in HS.

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Section: Discussionmentioning

confidence: 99%

Ektacytometry Analysis of Post-splenectomy Red Blood Cell Properties Identifies Cell Membrane Stability Test as a Novel Biomarker of Membrane Health in Hereditary Spherocytosis

et al. 2021

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“…However, young children without a spleen are at increased risk for infection. Therefore, PSE was used as another potential option and was proven a safe and effective alternative to splenectomy [ 35 ].…”

Section: Indications Of Psementioning

confidence: 99%

Clinical Application of Partial Splenic Embolization

Guan

2014

The Scientific World Journal

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Partial splenic embolization (PSE) is one of the intra-arterial therapeutic approaches of diseases. With the development of interventional radiology, the applications of PSE in clinical practice are greatly extended, while various materials are developed for embolization use. Common indications of PSE include hypersplenism with portal hypertension, hereditary spherocytosis, thalassemia, autoimmune hemolytic anemia, splenic trauma, idiopathic thrombocytopenic purpura, splenic hemangioma, and liver cancer. It is also performed to exclude splenic artery aneurysms from the parent vessel lumen and prevent aneurysm rupture, to treat splenic artery steal syndrome and improve liver perfusion in liver transplant recipients, and to administer targeted treatment to areas of neoplastic disease in the splenic parenchyma. Indicators of the therapeutic effect evaluation of PSE comprise blood routine test, changes in hemodynamics and in splenic volume. Major complications of PSE include the pulmonary complications, severe infection, damages of renal and liver function, and portal vein thrombosis. The limitations of PSE exist mainly in the difficulties in selecting the arteries to embolize and in evaluating the embolized volume.

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“…These reports are limited by small sample size, single-institution experience, and relatively short-term follow-up data [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27]. The goal of this study was to combine the experience and outcomes data from several large North American children's hospitals offering partial splenectomy in HS patients to assess efficacy and safety of partial splenectomy.…”

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confidence: 99%