Insights into determinants of spleen injury in sickle cell anemia

Hoss, Sara El; Cochet, Sylvie; Marin, Mickaël; Lapouméroulie, Claudine; Dussiot, Michaël; Bouazza, Naïm; Elie, Caroline; Montalembert, Mariane de; Arnaud, Cécile; Guitton, Corinne; Pellegrino, B.; Odièvre, Marie Hélène; Moati, F.; Kim, Caroline Le Van; Colin, Yves; Nemer, Wassim El; Brousse, Valentine

doi:10.1182/bloodadvances.2019000106

Cited by 31 publications

(34 citation statements)

References 36 publications

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“…This finding is corroborated by reports from the Middle East, which also showed preservation of spleen function into adulthood among patients with SCD with splenomegaly 73,92 . However, in contrast reports from the West suggest loss of splenic function in most patients by the age of 2 years 6,93,94 . In view of these conflicting data across regions, further studies are needed to fill this gap in knowledge regarding the age and prevalence of splenic dysfunction in patients with SCD in Africa.…”

Section: Discussionsupporting

confidence: 67%

The spectrum of splenic complications in patients with sickle cell disease in Africa: a systematic review

et al. 2020

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Summary The majority of the global population of sickle cell disease (SCD) patients resides in Africa. Individuals with this condition are at great risk of serious infections and early mortality secondary to splenic dysfunction without preventative measures. This review investigated the spectrum of splenic complications encountered in SCD among populations in Africa. We systematically searched several databases for all articles published through March 3, 2020. We included 55 studies from 14 African countries. This review reveals the difference in frequency of splenic complications in SCD in Africa when compared with their counterparts in the United State and Europe. While several studies (n = 45) described splenomegaly with a prevalence of 12% to 73% among children, and 4% to 50% among adults with HbSS, the reported prevalence for acute splenic sequestration crisis (n = 6 studies) and hypersplenism (n = 4 studies) was <10% and <5% respectively. A total of 30 surgical splenectomy was reported across eight studies. Only two (3.7%) studies provided data on spleen function. A conflicting pattern was observed amongst studies that evaluated the relationship between splenomegaly and the presence of bacterial and malaria infections. This review reveals the paucity of studies describing the role of SCD‐induced splenic dysfunction in morbidity and infection related mortality in Africa.

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Section: Discussionsupporting

confidence: 67%

The spectrum of splenic complications in patients with sickle cell disease in Africa: a systematic review

et al. 2020

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“…The red pulp is instead congested by RBCs, and congestion is probably caused by markedly impaired deformability and increased adhesion properties of sickle RBCs. These abnormalities play a key role in progressive spleen damage and loss of function [3].…”

Section: Introductionmentioning

confidence: 99%

“…A recent study using 99 m Technetium (Tc) sulfur-colloid liver–spleen scan to evaluate the phagocytic ability of splenic macrophages in 193 infants aged 8–18 months found that loss of splenic function began before 12 months of age in 86% of infants [4]. Another study, investigating the mechanical filtration ability of the spleen by using 99 m Tc heated RBC scintigraphy in a cohort of 57 infants with sickle cell anemia, showed that in 32% and 50% of infants, splenic function was decreased at 6 and 18 months of age, respectively [3]. The age varies for irreversible fibrosis of the spleen (a loss also referred to as autosplenectomy), and changes have been described over time, likely due to improved supportive care and the use of acute and chronic transfusion therapy [5].…”

Section: Introductionmentioning

confidence: 99%

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Serum Immunoglobulin Levels in Children with Sickle Cell Disease: A Large Prospective Study

Cherif-Alami

Hau

Kamdem

et al. 2019

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Over the past 3 decades, the pediatric department of the university Intercommunal Créteil hospital, a referral center for sickle cell disease (SCD), has prospectively evaluated immunoglobulin (Ig) levels in a cohort of 888 children with SCD, including 731 with severe sickle genotypes (HbSS and HbSβ0 thalassemia) and 157 with milder genotypes (HbSC and HbSβ+ thalassemia). We found consistent sickle genotype differences in levels of IgG and IgA, with increased levels of IgA and IgG in the severe versus milder genotype, from early childhood to late adolescence. Additionally, our results revealed a low serum IgM level, irrespective of sickle genotype. Finally, we found that IgA and IgG levels were significantly increased after therapeutic intensification with hydroxyurea but were stabilized in children receiving a transfusion program. The mechanisms contributing to these changes in Ig levels are unclear as is their clinical significance. We believe they should be further investigated.

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“…Measuring splenic function is of clinical interest in multiple conditions, including sickle cell anaemia (SCA) ( Brousse et al, 2014 ). In SCA, spleen damage occurs in early childhood, leading to loss of function within the first years of life ( Pearson et al, 1969 ; Pearson et al, 1985 ; El Hoss et al, 2019 ).…”

Section: Introductionmentioning

confidence: 99%

Automating Pitted Red Blood Cell Counts Using Deep Neural Network Analysis: A New Method for Measuring Splenic Function in Sickle Cell Anaemia

et al. 2022

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The spleen plays an important role in the body’s defence against bacterial infections. Measuring splenic function is of interest in multiple conditions, including sickle cell anaemia (SCA), where spleen injury occurs early in life. Unfortunately, there is no direct and simple way of measuring splenic function, and it is rarely assessed in clinical or research settings. Manual counts of pitted red blood cells (RBCs) observed with differential interference contrast (DIC) microscopy is a well-validated surrogate biomarker of splenic function. The method, however, is both user-dependent and laborious. In this study, we propose a new automated workflow for counting pitted RBCs using deep neural network analysis. Secondly, we assess the durability of fixed RBCs for pitted RBC counts over time. We included samples from 48 children with SCA and 10 healthy controls. Cells were fixed in paraformaldehyde and examined using an oil-immersion objective, and microscopy images were recorded with a DIC setup. Manual pitted RBC counts were performed by examining a minimum of 500 RBCs for pits, expressing the proportion of pitted RBCs as a percentage (%PIT). Automated pitted RBC counts were generated by first segmenting DIC images using a Zeiss Intellesis deep learning model, recognising and segmenting cells and pits from background. Subsequently, segmented images were analysed using a small ImageJ macro language script. Selected samples were stored for 24 months, and manual pitted RBC counts performed at various time points. When comparing manual and automated pitted RBC counts, we found the two methods to yield comparable results. Although variability between the measurements increased with higher %PIT, this did not change the diagnosis of asplenia. Furthermore, we found no significant changes in %PIT after storing samples for up to 24 months and under varying temperatures and light exposures. We have shown that automated pitted RBC counts, produced using deep neural network analysis, are comparable to manual counts, and that fixed samples can be stored for long periods of time without affecting the %PIT. Automating pitted RBC counts makes the method less time consuming and results comparable across laboratories.

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Insights into determinants of spleen injury in sickle cell anemia

Cited by 31 publications

References 36 publications

The spectrum of splenic complications in patients with sickle cell disease in Africa: a systematic review

The spectrum of splenic complications in patients with sickle cell disease in Africa: a systematic review

Serum Immunoglobulin Levels in Children with Sickle Cell Disease: A Large Prospective Study

Automating Pitted Red Blood Cell Counts Using Deep Neural Network Analysis: A New Method for Measuring Splenic Function in Sickle Cell Anaemia

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