Coexistence of sickle cell disease and systemic lupus erythematosus is associated with quantitative and qualitative impairments in circulating regulatory B cells

Boulassel, Mohamed-Rachid; Al-Naamani, Amal; Al-Zubaidi, Abeer; Al-Qarni, Zahra; Khan, Hammad; Oukil, Amar; Al-Badi, Amira; Alkaabi, Juma; Alshekaili, Jalila; Al-Hashmi, Sulaiman; Zadjali, Fahad; Qureshi, Rizwan; Panjwani, Vinodh; Alkindi, Salam

doi:10.1016/j.humimm.2022.09.005

Cited by 3 publications

(4 citation statements)

References 39 publications

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“…The incidence of connective tissue diseases such as SLE in adult patients with SCD appears to be increasing. The exact causes underlying this increased risk are still unknown, but a link with B regulatory (Breg) cells is possible as these cells suppress inflammatory responses and maintain tolerance [ 6 ]. Few published case reports have linked pericardial effusion as a rare complication in SCD [ 7 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

Cardiac Tamponade in Concurrent Sickle Cell Disease and Systemic Lupus Erythematosus: An Unusual Association

Elsaygh,

Liu,

Zaher

et al. 2024

Cureus

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This case report describes a rare occurrence of the coexistence of sickle cell disease (SCD) and systemic lupus erythematosus (SLE) in a 33-year-old female. The overlapping clinical manifestations posed diagnostic challenges, leading to a delayed diagnosis. The patient's presentation with pericardial effusion and tamponade during a concurrent SLE flare highlights the complexity of managing these conditions. The case underscores the importance of heightened clinical awareness and multidisciplinary collaboration for accurate diagnosis and timely intervention in such rare comorbidities.

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Section: Discussionmentioning

confidence: 99%

Cardiac Tamponade in Concurrent Sickle Cell Disease and Systemic Lupus Erythematosus: An Unusual Association

Elsaygh,

Liu,

Zaher

et al. 2024

Cureus

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“…Flow‐count fluorospheres (Beckman Coulter, USA) and standard fluorescent beads Megamix (BioCytex, France) were used to determine the absolute counts, and setup the flow cytometry with constant and known size regions for MP, respectively. Immunostaining was performed as previously reported 27 . In brief, plasma samples were stained with monoclonal antibodies, and incubated for 20 min in dark.…”

Section: Methodsmentioning

confidence: 99%

“…Immunostaining was performed as previously reported. 27 In brief, plasma samples were stained with monoclonal antibodies, and incubated for 20 min in dark. A binding buffer and flow-count fluorospheres, along with phosphate buffered saline were added to the sample and mounted for flow cytometry analysis.…”

Section: Reagents and Staining Proceduresmentioning

confidence: 99%

Impact of splenectomy on circulating microparticles in patients with sickle cell anemia

Boulassel,

Al‐Rubkhi,

Al‐Qasabi

et al. 2023

Int J Lab Hematology

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IntroductionCirculating microparticles (MP) are being described as potential biomarkers for disease activity in a variety of conditions including sickle cell anemia (SCA). However, relatively little is known about the influence of spleen status on MP levels in patients with SCA.MethodsUsing a prospective study design we characterize circulating MP in 144 patients with SCA in steady state by assessing their cellular origin and their relationships to spleen status defined by clinical and imaging findings. In addition, MP levels were studied according to demographic characteristics, clinical status, treatment modalities, and other hematological and biochemical parameters. Absolute plasma concentrations of MP were determined by flow cytometry.ResultsPatients with SCA displayed a 10‐fold increase in levels of MP derived from red blood cell (RBC) and platelets (PLT) when compared to their healthy counterparts (p < 0.0001). Splenectomized patients with SCA have more pronounced levels of MPRBC and MPPLT, and remained elevated after several weeks of follow‐up. Levels of MP were not significantly associated with spleen removal procedures, age, gender, clinical severity score, hydroxyurea therapy, hemoglobin F, and co‐existence of glucose‐6‐phosphate dehydrogenase deficiency.ConclusionCollectively, these results suggest that splenectomy affects circulating levels of MP regardless of the known SCA modifiers and correlates.

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“…They reported that in patients with auto-splenectomy, the mean CD4+ count was not significantly different to HbS patients with a normal-sized spleen. Several studies have further investigated the effect of hydroxyurea (HU) on lymphocyte subset counts [ 45 , 51 , 52 ] and have shown that SCD patients receiving HU had lower total lymphocytes, T cells, CD4+ T cells, memory CD4+ T cells, and memory CD8+ T cells compared to those who were untreated [ 24 ]. These findings may be explained by the fact that HU is often used as a chemotherapeutic drug and acts by inhibiting DNA synthesis, resulting in cytotoxicity and cell death.…”

Section: Immune Mechanisms Involved In the Pathogenesis Of Sickle Cel...mentioning

confidence: 99%

A Review of the Relationship between the Immune Response, Inflammation, Oxidative Stress, and the Pathogenesis of Sickle Cell Anaemia

Aboderin,

Oduola,

Davison

et al. 2023

Biomedicines

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Sickle cell anaemia (SCD) is a life-threatening haematological disorder which is predominant in sub-Saharan Africa and is triggered by a genetic mutation of the β-chain haemoglobin gene resulting in the substitution of glutamic acid with valine. This mutation leads to the production of an abnormal haemoglobin molecule called haemoglobin S (HbS). When deoxygenated, haemoglobin S (HbS) polymerises and results in a sickle-shaped red blood cell which is rigid and has a significantly shortened life span. Various reports have shown a strong link between oxidative stress, inflammation, the immune response, and the pathogenesis of sickle cell disease. The consequence of these processes leads to the development of vasculopathy (disease of the blood vessels) and several other complications. The role of the immune system, particularly the innate immune system, in the pathogenesis of SCD has become increasingly clear in recent years of research; however, little is known about the roles of the adaptive immune system in this disease. This review examines the interaction between the immune system, inflammation, oxidative stress, blood transfusion, and their effects on the pathogenesis of sickle cell anaemia.

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Coexistence of sickle cell disease and systemic lupus erythematosus is associated with quantitative and qualitative impairments in circulating regulatory B cells

Cited by 3 publications

References 39 publications

Cardiac Tamponade in Concurrent Sickle Cell Disease and Systemic Lupus Erythematosus: An Unusual Association

Cardiac Tamponade in Concurrent Sickle Cell Disease and Systemic Lupus Erythematosus: An Unusual Association

Impact of splenectomy on circulating microparticles in patients with sickle cell anemia

A Review of the Relationship between the Immune Response, Inflammation, Oxidative Stress, and the Pathogenesis of Sickle Cell Anaemia

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