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Plasma PTX3 levels in sickle cell disease patients, during vaso occlusion and acute chest syndrome (data from Saudi population).

Abstract

BACKGROUND

Sickle cell disease (SCD) is a chronic, incurable hereditary disease. The vaso-occlusive crisis (VOC) is the most frequently occurring acute complication in sickle cell patients and accounts for the majority of SCD-related hospital admissions. Another major complication is the potentially fatal acute chest syndrome (ACS). The prototypic long pentraxin-3 (PTX3), an acute phase protein and a key component of innate immunity, is linked to ischemia-induced inflammation, a condition incriminated in SCD complications.

AIM

To investigate the expression of PTX3 in stable SCD and VOC patients and to assess its relation to the development and progression of ACS.

SUBJECTS AND METHODS

We conducted this study on 160 patients with confirmed SCD (20 stable SCD and 140 in VOC), and 10 healthy age- and sex-matched controls. Patients were diagnosed as SCD by high-performance liquid chromatography. PTX3 levels were assessed using enzyme-linked immunosorbant assay.

RESULTS

In the stable state, all 20 SCD patients had PTX3 levels (range = 0.9-2.1 ng/ml; median = 1.1) comparable to those of healthy controls (range = 0.8-2.0 ng/ml; median = 1.0) (P > 0.05). During the VOC, plasma PTX3 significantly increased (range = 8.7-37.2 ng/ml; median = 22.3) (P < 0.01). Out of 140 VOC patients, 15 (10.7%) developed ACS and four required mechanical ventilation, of which two died. The median plasma level of PTX3 (22.3 ng/ml) was set as a cut-off value to stratify patients into low- and high-PTX3 expressers. Of the 140 VOC patients, 43 (30.7%) had PTX3 levels >22.3 ng/ml, of these, 13 patients developed ACS (13/43; 30.2%); of the remaining 97 patients who had PTX3 ≤22.3 ng/ml, only two patients (2/97; 2.1%) progressed to ACS, with a further increment in PTX3 in all of them. PTX3 levels were correlated with length of hospital stay in VOC patients and markers of lung injury in ACS patients.

CONCLUSION

PTX3 levels were higher in SCD patients in VOC, being associated with longer hospital stay. Higher initial PTX3 concentrations were related to the development of ACS with a further increase in PTX3 levels observed upon progression to ACS. Thus, PTX3 could be used as a subjective method to predict occurrence and severity of SCD acute complications.

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    MeSH

    Acute Chest Syndrome
    Adolescent
    Adult
    Anemia, Sickle Cell
    Biomarkers
    C-Reactive Protein
    Female
    Humans
    Male
    Middle Aged
    Predictive Value of Tests
    Saudi Arabia
    Serum Amyloid P-Component
    Young Adult

    Pub Type(s)

    Journal Article

    Language

    eng

    PubMed ID

    23735470

    Citation

    Elshazly, Shereen A., et al. "Plasma PTX3 Levels in Sickle Cell Disease Patients, During Vaso Occlusion and Acute Chest Syndrome (data From Saudi Population)." Hematology (Amsterdam, Netherlands), vol. 19, no. 1, 2014, pp. 52-9.
    Elshazly SA, Heiba NM, Abdelmageed WM. Plasma PTX3 levels in sickle cell disease patients, during vaso occlusion and acute chest syndrome (data from Saudi population). Hematology. 2014;19(1):52-9.
    Elshazly, S. A., Heiba, N. M., & Abdelmageed, W. M. (2014). Plasma PTX3 levels in sickle cell disease patients, during vaso occlusion and acute chest syndrome (data from Saudi population). Hematology (Amsterdam, Netherlands), 19(1), pp. 52-9. doi:10.1179/1607845413Y.0000000092.
    Elshazly SA, Heiba NM, Abdelmageed WM. Plasma PTX3 Levels in Sickle Cell Disease Patients, During Vaso Occlusion and Acute Chest Syndrome (data From Saudi Population). Hematology. 2014;19(1):52-9. PubMed PMID: 23735470.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Plasma PTX3 levels in sickle cell disease patients, during vaso occlusion and acute chest syndrome (data from Saudi population). AU - Elshazly,Shereen A, AU - Heiba,Nihal M, AU - Abdelmageed,Waleed M, Y1 - 2013/11/25/ PY - 2013/6/6/entrez PY - 2013/6/6/pubmed PY - 2014/10/10/medline KW - Acute chest syndrome KW - Pentraxin-3 KW - Sickle cell disease KW - Vaso-occlusive crisis SP - 52 EP - 9 JF - Hematology (Amsterdam, Netherlands) JO - Hematology VL - 19 IS - 1 N2 - BACKGROUND: Sickle cell disease (SCD) is a chronic, incurable hereditary disease. The vaso-occlusive crisis (VOC) is the most frequently occurring acute complication in sickle cell patients and accounts for the majority of SCD-related hospital admissions. Another major complication is the potentially fatal acute chest syndrome (ACS). The prototypic long pentraxin-3 (PTX3), an acute phase protein and a key component of innate immunity, is linked to ischemia-induced inflammation, a condition incriminated in SCD complications. AIM: To investigate the expression of PTX3 in stable SCD and VOC patients and to assess its relation to the development and progression of ACS. SUBJECTS AND METHODS: We conducted this study on 160 patients with confirmed SCD (20 stable SCD and 140 in VOC), and 10 healthy age- and sex-matched controls. Patients were diagnosed as SCD by high-performance liquid chromatography. PTX3 levels were assessed using enzyme-linked immunosorbant assay. RESULTS: In the stable state, all 20 SCD patients had PTX3 levels (range = 0.9-2.1 ng/ml; median = 1.1) comparable to those of healthy controls (range = 0.8-2.0 ng/ml; median = 1.0) (P > 0.05). During the VOC, plasma PTX3 significantly increased (range = 8.7-37.2 ng/ml; median = 22.3) (P < 0.01). Out of 140 VOC patients, 15 (10.7%) developed ACS and four required mechanical ventilation, of which two died. The median plasma level of PTX3 (22.3 ng/ml) was set as a cut-off value to stratify patients into low- and high-PTX3 expressers. Of the 140 VOC patients, 43 (30.7%) had PTX3 levels >22.3 ng/ml, of these, 13 patients developed ACS (13/43; 30.2%); of the remaining 97 patients who had PTX3 ≤22.3 ng/ml, only two patients (2/97; 2.1%) progressed to ACS, with a further increment in PTX3 in all of them. PTX3 levels were correlated with length of hospital stay in VOC patients and markers of lung injury in ACS patients. CONCLUSION: PTX3 levels were higher in SCD patients in VOC, being associated with longer hospital stay. Higher initial PTX3 concentrations were related to the development of ACS with a further increase in PTX3 levels observed upon progression to ACS. Thus, PTX3 could be used as a subjective method to predict occurrence and severity of SCD acute complications. SN - 1607-8454 UR - https://www.unboundmedicine.com/medline/citation/23735470/Plasma_PTX3_levels_in_sickle_cell_disease_patients_during_vaso_occlusion_and_acute_chest_syndrome__data_from_Saudi_population__ L2 - http://www.tandfonline.com/doi/full/10.1179/1607845413Y.0000000092 DB - PRIME DP - Unbound Medicine ER -