Serum erythropoietin levels in thalassemia intermedia

Dore, Fausto; Bonfigli, Silvana; Gaviano, E.; Pardini, Simonetta; Cianciulli, Paolo; Papa, G; Longinotti, M

doi:10.1007/bf01695865

Cited by 31 publications

(26 citation statements)

References 9 publications

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“…Similar values were also reported by Di Marco et al [16]. Several other studies have investigated the role of cytokines and interleukins in the pathophysiology of beta-thalassaemia [17][18][19][20][21][22].…”

Section: Discussionsupporting

confidence: 86%

Interleukins 12 and 13 levels among beta-thalassaemia major patients

Hashad

Hamed²,

Gharabawy³

et al. 2013

East Mediterr Health J

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The role of inflammatory cytokines in the pathophysiology of beta-thalassaemia is still unclear. In this study production levels of interleukins (IL)-12 and IL-13 were measured by commercial ELISA in culture supernatants of mitogen-stimulated peripheral blood mononuclear cells from 30 non-splenectomized betathalassaemia cases with iron overload and 20 age-and sex-matched healthy individuals. IL-12 levels were significantly lower among cases compared with controls (91.4 pg/mL versus 154.6 pg/mL), while IL-13 levels were significantly higher (42.5 pg/mL versus 5.7 pg/mL). There was a significant negative correlation between IL-12 and IL-13 levels among beta-thalassaemia cases (r = -0.42). Patients with beta-thalassaemia alone had higher IL-12 levels than beta-thalassaemia patients who were seropositive for chronic hepatitis B or C virus infection (140 pg/mL versus 50 pg/mL); IL-13 levels were slightly lower (65 pg/mL versus 67 pg/mL). An imbalance in the IL-12/IL-13 axis may be relevant to the pathophysiology of beta-thalassaemia. Taux des interleukines 12 et 13 chez des patients atteints de bêta-thalassémie majeureRÉSUMÉ Le rôle des cytokines inflammatoires dans la physiopathologie de la bêta-thalassémie reste à élucider. Dans la présente étude, les taux de production des interleukines 12 et 13 (IL-12 et IL-13) ont été mesurés à l'aide de la méthode ELISA dans les surnageants de culture de cellules mononucléées de sang périphérique stimulées par des mitogènes chez 30 cas de bêta-thalassémie non splénectomisés présentant une surcharge martiale ainsi que chez 20 individus en bonne santé appariés pour l'âge et le sexe. Les taux d'IL-12 étaient nettement inférieurs chez les malades par rapport aux témoins (91,4 pg/ml contre 154,6 pg/ml), alors que les taux d'IL-13 étaient significativement plus élevés (42,5 pg/ml contre 5,7 pg/ml). Une corrélation négative importante existait entre les taux d'IL-12 et d'IL-13 chez les patients atteints de beta-thalassémie (r = -0,42). Les patients uniquement porteurs d'une bêta-thalassémie avaient des taux d'IL-12 plus élevés que les patients atteints d'une bêta-thalassémie et également chroniquement infectés par le virus de l'hépatite B ou C (140 pg/ml contre 50 pg/ml) ; les taux de l'IL-13 étaient légèrement inférieurs (65 pg/ml contre 67 pg/ml). Un défaut de l'axe IL-12/IL-13 peut être révélateur de la physiopathologie de la bêta-thalassémie.

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Section: Discussionsupporting

confidence: 86%

Interleukins 12 and 13 levels among beta-thalassaemia major patients

Hashad

Hamed²,

Gharabawy³

et al. 2013

East Mediterr Health J

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“…1 These clinical features have also been reported in thalassemia intermedia, hereditary spherocytosis, and other forms of severe hemolytic anemia. [47][48][49][50][51][52][53][54][55][56][57][58][59][60][61][62] This suggests that sickling is not required for this subphenotype and instead implicates the severe hemolysis of sickle cell disease in its pathobiology. Consistent with this model, the Jamaican sickle cell population, characterized by severe hemolysis, has a much higher prevalence of leg ulcers and priapism than those in Greece and India, who have less severe hemolysis, but comparable rates of VOC pain crisis.…”

Section: Discussionmentioning

confidence: 99%

Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease

Kato

McGowan

Machado

et al. 2006

Blood

561

346

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Pulmonary hypertension is prevalent in adult patients with sickle cell disease and is strongly associated with early mortality and markers of hemolysis, in particular, serum lactate dehydrogenase (LDH). Intravascular hemolysis leads to impaired bioavailability of nitric oxide (NO), mediated by NO scavenging by plasma oxyhemoglobin and by arginine degradation by plasma arginase. We hypothesized that serum LDH may represent a convenient biomarker of intravascular hemolysis and NO bioavailability, characterizing a clinical subphenotype of hemolysisassociated vasculopathy. In a cohort of 213 patients with sickle cell disease, we found statistically significant associations of steady-state LDH with low levels of hemoglobin and haptoglobin and high levels of reticulocytes, bilirubin, plasma hemoglobin, aspartate aminotransferase, arginase, and soluble adhesion molecules. LDH isoenzyme fractionation confirmed predominance of LD1 and LD2, the principal isoforms within erythrocytes. In a subgroup, LDH levels closely correlated with plasma cell-free hemoglobin, accelerated NO consumption by plasma, and impaired vasodilatory responses to an NO donor. Remarkably, this simple biomarker was associated with a clinical subphenotype of pulmonary hypertension, leg ulceration, priapism, and risk of death in patients with sickle cell disease. We propose that LDH elevation identifies patients with a syndrome of hemolysisassociated NO resistance, endothelial dysfunction, and end-organ vasculopathy. IntroductionPulmonary hypertension is an increasingly recognized complication of sickle cell disease and other chronic hereditary and acquired hemolytic anemias. Doppler echocardiography screening reveals a tricuspid regurgitant jet velocity (TRV) of 2.5 m/s or greater in 33% of adults with sickle cell disease, indicative of pulmonary hypertension. 1 Pulmonary hypertension in these patients is associated with a 10-fold increased risk for early mortality. Elevated pulmonary artery pressures in patients with sickle cell disease have been associated with low hemoglobin concentration, high levels of serum lactate dehydrogenase (LDH), elevated systolic systemic blood pressure, history of priapism, renal insufficiency, and markers of iron overload. Of the several independent epidemiologic factors associated with pulmonary hypertension, an elevated level of serum LDH has drawn particular interest and controversy. In addition to a link with pulmonary hypertension, we and others have observed a link between LDH and a generalized state of endothelial activation, reflected by elevated blood plasma levels of soluble adhesion molecules, especially vascular cell adhesion molecule (VCAM-1). 2-10 Both pathologic endothelial activation and pulmonary hypertension are associated with more severe hemolytic anemia, reflected by low hemoglobin levels and high reticulocyte counts. 1,2,8 These data begin to suggest that LDH elevation may be a marker of hemolysis-associated endothelial dysfunction and pulmonary hypertension.LDH has long been considered a useful c...

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“…Indirect evidence of increased and prolonged tissue injury in thalassemic and sickle patients includes activation of polymorphonuclear neutrophils and monocytes and the increased levels of neutrophil elastase and circulating cytokines. 49,64,[69][70][71][72] It should be stated that of all hemoglobinopathies, ␤ thalassemia is the most informative in terms of elastic tissue abnormalities. Extensive clinical and histologic findings have been presented in the literature.…”

Section: Pathogenesis and Geneticsmentioning

confidence: 99%

Elastic tissue abnormalities resembling pseudoxanthoma elasticum in β thalassemia and the sickling syndromes

Aessopos

Farmakis

Loukopoulos

2002

Blood

139

124

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The development of clinical and histopathologic manifestations of a diffuse elastic tissue defect, resembling inherited pseudoxanthoma elasticum (PXE), has been encountered with a notable frequency in patients with ␤ thalassemia, sickle cell disease, and sickle thalassemia. The PXE-like clinical syndrome, consisting of skin, ocular, and vascular manifestations, has a variable severity in these hemoglobinopathies and it is age-dependent, with a generally late onset, after the second decade of life. The defect is believed to be acquired rather than inherited and related to the consequences of the primary disease. The high prevalence of the findings implicates the elastic tissue injury as one of the main comorbid abnormalities encountered in ␤ thalassemia and the sickling syndromes. In these patients a number of complications, sometimes serious, has been recognized to be related to ocular and vascular elastic tissue defects. Because several organ systems are involved, each medical specialty should be aware of the phenomenon. This coexistence, on the other hand, introduces a novel pathogenetic aspect of PXE and an important research challenge. (Blood. 2002;99:30-35)

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Serum erythropoietin levels in thalassemia intermedia

Cited by 31 publications

References 9 publications

Interleukins 12 and 13 levels among beta-thalassaemia major patients

Interleukins 12 and 13 levels among beta-thalassaemia major patients

Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease

Elastic tissue abnormalities resembling pseudoxanthoma elasticum in β thalassemia and the sickling syndromes

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