Pocked erythrocyte counts in patients with hereditary spherocytosis before and after splenectomy

Buchanan, George R.; Holtkamp, Christine A.

doi:10.1002/ajh.2830250304

Cited by 5 publications

(5 citation statements)

References 13 publications

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“…The present simulations do not account for the effect of a putatively impaired splenic RBC filtration function in HS patients ( 15 , 36 ). Therefore, the prediction of RBC retention in Figs.…”

Section: Discussionmentioning

confidence: 93%

Mechanics of diseased red blood cells in human spleen and consequences for hereditary blood disorders

et al. 2018

Proc. Natl. Acad. Sci. U.S.A.

112

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SignificanceThe interendothelial slit (IES) is the narrowest circulatory pathway in the human spleen where aged and diseased red blood cells (RBCs) are filtered. We use a two-component RBC model to probe the dynamics of healthy and diseased RBCs traversing IES. Our simulations reveal that the spleen not only senses and clears RBCs with abnormal shapes and deformability but also alters the geometries of RBCs that contain protein defects arising from hereditary blood disorders. The framework presented here is sufficiently general to be extended to elucidate the pathophysiological roles of the spleen in other blood diseases.

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Section: Discussionmentioning

confidence: 93%

Mechanics of diseased red blood cells in human spleen and consequences for hereditary blood disorders

et al. 2018

Proc. Natl. Acad. Sci. U.S.A.

112

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“…by guest www.bloodjournal.org From either HS or P falciparum malaria. Although the clearance of mechanically altered, surface altered, and normal RBCs is generally accelerated in P falciparum-infected adults, [32][33][34] splenic RBC filtration function is partially impaired in HS patients, as illustrated by the abnormally high proportion of circulating "pocked" RBCs 35 and the occasional observation of Howell-Jolly bodies on the blood smears of HS patients. 35 Therefore, the different retention rates of freshly collected HS-RBCs and ring-RBCs in microbeads may result from the fact that the spleen of HS patients leaves a proportion of poorly deformable HS-RBCs in circulation, although the spleen of malaria patients rapidly retains most poorly deformable ring-RBCs.…”

Section: Spleen-like Sensing Of Rigid Erythrocytes E93mentioning

confidence: 99%

“…Although the clearance of mechanically altered, surface altered, and normal RBCs is generally accelerated in P falciparum-infected adults, [32][33][34] splenic RBC filtration function is partially impaired in HS patients, as illustrated by the abnormally high proportion of circulating "pocked" RBCs 35 and the occasional observation of Howell-Jolly bodies on the blood smears of HS patients. 35 Therefore, the different retention rates of freshly collected HS-RBCs and ring-RBCs in microbeads may result from the fact that the spleen of HS patients leaves a proportion of poorly deformable HS-RBCs in circulation, although the spleen of malaria patients rapidly retains most poorly deformable ring-RBCs. The modification of spleen-filtering function in HS patients is mild compared with that of patients with severe functional or anatomic asplenia, 35,36 probably explaining why putative impaired RBC retention is not associated with an increased risk of overwhelming pneumococcal infection in HS (as opposed to what occurs in splenectomized subjects).…”

Section: Spleen-like Sensing Of Rigid Erythrocytes E93mentioning

confidence: 99%

“…35 Therefore, the different retention rates of freshly collected HS-RBCs and ring-RBCs in microbeads may result from the fact that the spleen of HS patients leaves a proportion of poorly deformable HS-RBCs in circulation, although the spleen of malaria patients rapidly retains most poorly deformable ring-RBCs. The modification of spleen-filtering function in HS patients is mild compared with that of patients with severe functional or anatomic asplenia, 35,36 probably explaining why putative impaired RBC retention is not associated with an increased risk of overwhelming pneumococcal infection in HS (as opposed to what occurs in splenectomized subjects). Taken together, these observations suggest that hyposplenism is not an all-or-nothing phenomenon and that new markers, like the retention rate of circulating RBC in microbeads, would help delineate its heterogeneity in diverse clinical situations.…”

Section: Spleen-like Sensing Of Rigid Erythrocytes E93mentioning

confidence: 99%

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The sensing of poorly deformable red blood cells by the human spleen can be mimicked in vitro

Deplaine

Safeukui

Jeddi

et al. 2011

Blood

181

245

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Retention of poorly deformable red blood cells (RBCs IntroductionRetention of poorly deformable red blood cells (RBCs) by the human spleen has been recognized as a critical determinant of pathogenesis of several RBC disorders, including hereditary spherocytosis and malaria. The severity of hereditary spherocytosis (HS), a syndrome resulting from mutations of RBC membrane proteins, 1 is related to the extent of decreased membrane surface area. Splenic retention is the dominant mechanism responsible for reduced life span of HS-RBCs, such that splenectomy is beneficial in reducing anemia in symptomatic HS patients. 1 Changes of RBC mechanical properties have also been observed in blood stored for 3 weeks or longer. 2 Prolonged storage influences the survival of RBCs after transfusion 3 and probably contributes to transfusion-related side effects, including respiratory distress and systemic sepsis. [3][4][5] Not least, the deformability of Plasmodium falciparum-infected red blood cells (Pf-RBCs) is also central in malaria pathogenesis. It progressively decreases as P falciparum matures in its host RBCs, from rings (ring-RBCs) 6 to trophozoites and then to schizonts. 7,8 When mature stages are sequestered in small vessels, they escape retention in the spleen. Ring-RBCs only rarely adhere to endothelial cells 9 and are found in the peripheral blood circulation. 10,11 We recently showed that a fraction of ring-RBCs is retained in isolated-perfused human spleens. 12 Ring-RBCs accumulated upstream from interendothelial slits, a spleen-specific microanatomic structure that retains RBCs with reduced deformability (rigid-RBCs). [13][14][15] In brief, in malaria as in several other RBC disorders, extensive RBC retention in the spleen is associated with anemia, 1,16,17 whereas impaired retention potentially favors adverse events, such as acute microvascular obstruction in malaria patients, 18,19 late vascular disorders in splenectomized patients with HS or thalassemia, 20,21 and acute organ dysfunction in critically ill patients transfused with "older" blood. 4 Exploration of how the human spleen senses poorly deformable RBCs would be facilitated by a physiologically relevant in vitro approach simpler than the ex vivo spleen perfusion system. To experimentally replicate the mechanical sensing of RBC by the splenic microcirculation, we first analyzed the fine structure of interendothelial slits in the human spleen. We realized that the geometry of narrow and short interendothelial slits would be accurately mimicked by spaces between microbeads. We then allowed RBCs to flow through a The publication costs of this article were defrayed in part by page charge payment. Therefore, and solely to indicate this fact, this article is hereby marked ''advertisement'' in accordance with 18 USC section 1734. For personal use only. on April 8, 2019. by guest www.bloodjournal.org From mixture of 5-to 25-m-diameter microbeads. Heated RBCs, Pf-RBCs, and RBCs from patients with HS were retained in the microbead layer without hemolysis. The r...

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“…Although the percentage of pitted erythrocytes has been determined in different haemolytic states (4, 5, [9][10][11][12], the preferential localisation of pits in favour of a certain morphological population of RBCs has not been reported previously, while the preferential localisation of red cell vacuoles to old RBCs was shown by different approaches (13, 14). Thus the preferential localisation of vacuoles to pathologically shaped erythrocytes (acanthocytes, schizocytes and even elliptocytes) appears to be new morphological sign in hyposplenism with practical implications.…”

Section: Discussionmentioning

confidence: 99%

Preferential localisation of red cell vacuoles to pathologically shaped human red blood cells

Demeter

Magyar

Varga

et al. 1989

European J of Haematology

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The percentage of pitted erythrocytes and Howell‐Jolly bodies in peripheral blood samples of 51 individuals following posttraumatic splenectomy and 20 patients splenectomized because of various haematological diseases differed significantly from each other (p < 0.001) and from that of healthy controls (p < 0.001). The percentage of pitted erythrocytes was significantly higher in pathologically shaped red blood cells (RBCs) (acanthocytes, schizocytes, elliptocytes) than in normal discoid shaped RBCs (p < 0.001). As the number of pits per RBC showed great individual variations, a scoring system for the evaluation of pitted RBCs is proposed.

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Pocked erythrocyte counts in patients with hereditary spherocytosis before and after splenectomy

Cited by 5 publications

References 13 publications

Mechanics of diseased red blood cells in human spleen and consequences for hereditary blood disorders

Mechanics of diseased red blood cells in human spleen and consequences for hereditary blood disorders

The sensing of poorly deformable red blood cells by the human spleen can be mimicked in vitro

Preferential localisation of red cell vacuoles to pathologically shaped human red blood cells

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