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. 2023 Jan 13;4(1):211-215.
doi: 10.1002/jha2.643. eCollection 2023 Feb.

Predictors and clinical complications associated with antiphospholipid antibodies in sickle cell disease

Affiliations

Predictors and clinical complications associated with antiphospholipid antibodies in sickle cell disease

Claudia Rodriguez Rivera et al. EJHaem. .

Abstract

Although a higher prevalence of antiphospholipid autoantibodies (aPL) has been observed in some cohorts of sickle cell disease (SCD) patients, the clinical risk factors for the development of aPL and its associated complications remain unclear. In a retrospective study of 63 SCD patients, a lower hemoglobin concentration and higher white blood cell count were independently associated with an elevated aPL. SCD patients with elevated aPL had increased pregnancy complications (≥3 miscarriages, preterm delivery, pre-eclampsia) and venous thrombotic events. Our findings suggest that SCD may predispose to the generation of aPL and that aPL itself may contribute to the vasculopathy of SCD. Prospective testing for aPL is warranted in patients with SCD.

Keywords: antiphospholipid antibody; antiphospholipid syndrome; multiorgan failure; sickle cell disease; systemic lupus erythematosus.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

Figures

FIGURE 1
FIGURE 1
Association of antiphospholipid antibodies (aPL) with laboratory and clinical variables in patients with sickle cell disease (SCD). Patients with systemic lupus erythematosus (SLE) had higher (A) anti‐β2 glycoprotein I IgG and (B) anticardiolipin IgM levels and (C) a trend for higher anticardiolipin IgM levels compared to those without SLE. A lower hemoglobin concentration was associated with (D) a trend for higher anticardiolipin IgG levels and (E) the presence of a lupus anticoagulant. (F) Pregnancy complications and (G) venous thrombotic events were observed in a higher proportion of patients with an elevated aPL compared to those with a normal level. Blood counts in hospitalized SCD patients demonstrated (H) lower platelet and (I) a trend for higher white blood cell (WBC) counts in those with an elevated aPL and multiorgan failure (MOF), compared to those without MOF or MOF with a normal aPL; (J) no significant difference was observed for hemoglobin concentration

References

    1. Dragoni F, Iori AP, Pignoloni P, Minotti C, Chiarotti F, Mazzucconi MG, et al. Thrombophilic screening in patients with paroxysmal nocturnal haemoglobinuria: a pilot study. Br J Haematol. 2010;150(4):492–4. - PubMed
    1. Merashli M, Arcaro A, Graf M, Gentile F, Ames PRJ. Autoimmune haemolytic anaemia and antiphospholipid antibodies in paediatrics: a systematic review and meta‐analysis. Clin Rheumatol. 2021;40(5):1967–73. - PubMed
    1. Hannemann A, Rees DC, Brewin JN, Noe A, Low B, Gibson JS. Oxidative stress and phosphatidylserine exposure in red cells from patients with sickle cell anaemia. Br J Haematol. 2018;182(4):567–78. - PMC - PubMed
    1. Merashli M, Arcaro A, Graf M, Caruso M, Ames PRJ, Gentile F. Antiphospholipid antibodies in sickle cell disease: a systematic review and exploratory meta‐analysis. Clin Appl Thromb Hemost. 2021;27:10760296211002914. - PMC - PubMed
    1. Ruiz MAD, Shah BN, Ren G, Hussain F, Njoku F, Machado RF, et al. Haptoglobin 1 Allele predicts higher serum haptoglobin and lower multiorgan failure risk in sickle cell disease. Blood Adv. 2022;6:6242–8. - PMC - PubMed