Evaluation of reticulated platelets in patients with sickle cell diseases

Noronha, José F. A.; Costa, Fernando Ferreira; Saad, Sara Teresinha Olalla; Lorand-Metze, Irene; Grotto, Helena Zerlotti Wolf

doi:10.1016/j.thromres.2007.04.002

Cited by 13 publications

(9 citation statements)

References 20 publications

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“…22 The increased level of sPselectin (indicating platelet disturbances) during crisis suggests that it is a transient feature associated with acute events. Notably, platelet expression of P-selectin is raised in the veno-occlusive crisis of SCD, 21 and this supports our finding of raised soluble levels in our patients in crisis. However, in our study, as the increase in NTBI during crisis was not significant, the iron abnormality seems unlikely to contribute, in an acute setting, to raised sP-selectin alone.…”

Section: Discussionsupporting

confidence: 91%

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Increased levels of soluble P-selectin correlate with iron overload in sickle cell disease

Blann

Marwah

Cobley

et al. 2007

British Journal of Biomedical Science

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Homozygous sickle cell disease (SCD) is characterised by increased soluble P-selectin (sP-selectin), suggesting increased platelet activation, and high non-transferrin-bound iron (NTBI), reflecting iron overload, possibly due to blood transfusion. Hypothesising a relationship between these processes, we measured both markers in 40 SCD patients and 40 age/gender/race-matched controls, finding increased levels of each marker in the patients (both P<0.001), but more pertinently a significant NTBI/sP-selectin correlation (r=0.52, P<0.001). Both indices were increased in the blood of 15 recently-transfused patients compared with 25 three-month transfusion-free patients (P<0.001), but only sP-selectin was higher in present sickle crisis (P<0.001). We suggest that increased NTBI associated with blood transfusion iron overload in SCD may promote platelet activation.

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Section: Discussionsupporting

confidence: 91%

“…This study confirms the presence of raised plasma levels of sP-selectin 9,16,20,21 in SCD. Despite the widely held view that sP-selectin arises almost exclusively from platelets, 7 we acknowledge the possibility that some may arise from an activated endothelium, although some of this evidence is from transgenic sickle mice.…”

Section: Discussionsupporting

confidence: 86%

Increased levels of soluble P-selectin correlate with iron overload in sickle cell disease

Blann

Marwah

Cobley

et al. 2007

British Journal of Biomedical Science

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“…However, a study of 16 patients with steady state sickle cell anemia in the U.K. found decreased mean platelet volume in patients compared with ethnically matched controls (7.6 ± 1.1 vs. 8.4 ± 0.9) (67). Most recently, a study of 89 Brazilian patients with SCD (including patients SC and Sβ-thalassemia) found an increase in the total number of platelets larger than 12 fl, especially in patients with pain crisis, but the percentage of large platelets was not increased (52).Genetic, environmental, and/or technical variations may account for the reported differences in platelet size in patients with SCD. Additional studies of platelet size in humans with SCD, along with appropriate controls, are needed to clarify this issue.…”

Section: Discussionmentioning

confidence: 98%

“…The one parameter suggesting thrombopoietic stress was the increased thiazole orange staining of platelets. An increase in platelets staining with thiazole-orange has been reported in humans with SCD, with the greatest increase in patients during pain crisis (52). Elevated erythropoietin levels in SS mice and patients with SCD may contribute to the increase, since erythropoietin therapy in neonates has been associated with an increase in thiazole orange-staining platelets even without producing an increase in platelet count (60).…”

Section: Discussionmentioning

confidence: 99%

Morphological and functional platelet abnormalities in Berkeley sickle cell mice

Shet

Hoffmann

Jiroušková

et al. 2008

Blood Cells, Molecules, and Diseases

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Berkeley sickle cell mice are used as an animal model of human sickle cell disease but there are no reports of platelet studies in this model. Since humans with sickle cell disease have platelet abnormalities, we studied platelet morphology and function in Berkeley mice (SS). We observed elevated mean platelet forward angle light scatter (FSC) values (an indirect measure of platelet volume) in SS compared to wild type (WT) (37 ± 3.2 vs. 27 ± 1.4, mean ± SD; p <0.001), in association with moderate thrombocytopenia (505 ± 49 × 10 3 /μl vs. 1151 ± 162 × 10 3 /μl; p <0.001). Despite having marked splenomegaly, SS mice had elevated levels of Howell-Jolly bodies and "pocked" erythrocytes (p <0.001 for both) suggesting splenic dysfunction. SS mice also had elevated numbers of thiazole orange positive platelets (5 ± 1 % vs. 1 ± 1%; p <0.001), normal to low plasma thrombopoietin levels, normal plasma glycocalicin levels, normal levels of platelet recovery, and near normal platelet life spans. Platelets from SS mice bound more fibrinogen and antibody to Pselectin following activation with a threshold concentration of a protease activated receptor (PAR)-4 peptide compared to WT mice. Enlarged platelets are associated with a predisposition to arterial thrombosis in humans and some humans with SCD have been reported to have large platelets. Thus, additional studies are needed to assess whether large platelets contribute either to pulmonary hypertension or the large vessel arterial occlusion that produces stroke in some children with sickle cell disease.

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“…Noronha et al [55], demonstrated that patients with sickle cell vasoocclusive crisis had higher circulating RP vs patients in stable phase.…”

Section: Vaso-occlusive Crisis In Sickle Cell Anemiamentioning

confidence: 99%

Clinical applicability of reticulated platelets

Dusse

Freitas

2015

Clinica Chimica Acta

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Evaluation of reticulated platelets in patients with sickle cell diseases

Cited by 13 publications

References 20 publications

Increased levels of soluble P-selectin correlate with iron overload in sickle cell disease

Increased levels of soluble P-selectin correlate with iron overload in sickle cell disease

Morphological and functional platelet abnormalities in Berkeley sickle cell mice

Clinical applicability of reticulated platelets

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