RESUMEN
This century has seen a revolution the management of beta-thalassemia major. Over a 12-year period to 2016, we aimed to analyze the benefits of such advances. In 209 patients, independent of the chelation regimen, ferritin, cardiac T2* and liver iron concentration changes were evaluated. We defined chelation success (ChS) as no iron load in the heart and acceptable levels in the liver. Over 3 early magnetic resonance imagings, the same parameters were assessed in 2 subgroups, the only 2 that had sufficient patients continuing on 1 regimen and for a significant period of time, 1 on deferrioxamine (low iron load patients nâ =â 41, Group A) and 1 on deferoxamine-deferiprone (iron overloaded nâ =â 60, Group B). Finally, 28 deaths and causes were compared to those of an earlier period. The 209 patients significantly optimized those indices, while the number of patients with chelation success, increased from 6% to 51% (Pâ <â .0001). In group A, ChS after about 8 years increased from 21 to 46% (Pâ =â .006), while in Group B, from 0% to 60% (Pâ <â .001) after about 7 years. Deaths over the 2 periods showed significant reduction. Combined clearance of cardiac and liver iron (ChS) is feasible and should become the new target for all patients. This requires, serial magnetic resonance imagings and often prolonged intensified chelation for patients.
Asunto(s)
Quelantes del Hierro , Talasemia beta , Humanos , Quelantes del Hierro/uso terapéutico , Talasemia beta/tratamiento farmacológico , Deferoxamina/uso terapéutico , Deferiprona/uso terapéutico , Terapia por Quelación , Piridonas/uso terapéutico , Hierro/uso terapéutico , Hígado/diagnóstico por imagenRESUMEN
The category of myelodysplastic syndromes/myeloproliferative diseases (MDS/MPD) is a relatively new group of malignant hematologic diseases developed by the World Health Organization. These hematologic disorders lack the BCR/ABL fusion gene, although they can be associated with chromosomal translocations that involve genes encoding other protein kinases. Imatinib mesylate was recognized as a potent inhibitor of some of those kinases. We present a patient with a previously treated acute myeloid leukemia, who, after a 9-year-long remission, developed an MDS/MPD with normal karyotype, which initially responded to imatinib mesylate. Translocation t(12;13)(p12;q14) was detected after loss of response to imatinib treatment. Translocation t(12;13) is rare. It has been described in several hematologic malignancies including chronic myelomonocytic leukemia but not in MDS/MPD, previously described as Philadelphia-negative chronic myelogenous leukemia. Moreover, the correlation of this molecular abnormality with loss of efficacy of imatinib is unique in the literature.
Asunto(s)
Resistencia a Antineoplásicos , Proteínas de Fusión bcr-abl/metabolismo , Síndromes Mielodisplásicos/genética , Trastornos Mieloproliferativos/genética , Piperazinas/uso terapéutico , Inhibidores de Proteínas Quinasas/uso terapéutico , Pirimidinas/uso terapéutico , Translocación Genética , Antineoplásicos/uso terapéutico , Benzamidas , Femenino , Proteínas de Fusión bcr-abl/genética , Humanos , Mesilato de Imatinib , Persona de Mediana Edad , Síndromes Mielodisplásicos/tratamiento farmacológico , Trastornos Mieloproliferativos/tratamiento farmacológicoRESUMEN
Little is known about iron metabolism in skeletal muscle while hepatic iron metabolism is well understood. The aim of this study is to compare the iron metabolism gene expression profile in skeletal muscle and the liver in humans. Muscle and hepatic biopsies from six normal individuals were acquired. Twelve genes involved in iron metabolism( import, storage, export) were selected to be studied. Reverse transcriptase polymerase chain reaction (RT-PCR) was performed in order to determine the expression profile in skeletal muscle and compare it to the one from the liver. Semi-quantification of the gene expression in the studied tissues was performed by densitometric analysis (DA). The results were expressed relative to the percentage of the ß-actin gene. Fine analysis was performed by real-time PCR (q-PCR) quantification for the genes that their expression presented a difference of more than 20% in the 2 tissues in the first applied densitometric analysis. Most of the studied genes, HJV, TFR1, HFE, DMT1, DMT1nonIRE, NGAL, HEPH, IREG1, FTH1 were well expressed (>70% of ß-actin) in skeletal muscle . HAMP, CP, and TFR2 were absent or minimally expressed (<10% of ß-actin) in skeletal muscle while they were well expressed in liver. HJV and Heph were found to have higher expression in skeletal muscle (SM) compared to liver (L) (SM/L=2.65 ± 1.1(p<0.05) and SM/L=1.5 ± 0.06(p<0.05 respectively in q-PCR). The relative expressions of the studied genes in both tissues and their relative contribution in iron homeostasis in different pathways are discussed.
Asunto(s)
Regulación de la Expresión Génica/fisiología , Homeostasis/fisiología , Hierro/metabolismo , Proteínas Musculares/biosíntesis , Músculo Esquelético/metabolismo , Adulto , Femenino , Humanos , Hígado/metabolismo , Masculino , Especificidad de Órganos/fisiología , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa/métodosRESUMEN
The reproductive thalassemic population is growing older and doctors confront the challenge of the thalassemic pregnancy. Pregnancy is characterized by dynamic multiple system changes, resulting in increased basal oxygen consumption, changes in energy substrate use by different organs and increased susceptibility to oxidative stress, while homozygous transfusion-dependent beta-thalassemia (beta-thal) patients manifest cardiac, hepatic, endocrine, and metabolic disorders attributable to chronic anoxia and iron overload. Pregnant thalassemic patients require significantly larger amount of total blood transfusion during pregnancy and iron overload increases the oxidative stress of pregnancy, while the risk for cardiovascular events, in a high cardiac output state, is augmented and chelation treatment is generally avoided due to the potential teratogenicity. Pregnancy in thalassemia major should be considered high risk, and be cared for by an expert team with special caution and sensitivity.
Asunto(s)
Sistema Cardiovascular/fisiopatología , Sobrecarga de Hierro/fisiopatología , Complicaciones Cardiovasculares del Embarazo/fisiopatología , Complicaciones Hematológicas del Embarazo/fisiopatología , Talasemia beta/fisiopatología , Femenino , Humanos , Embarazo , Factores de Riesgo , Sensibilidad y EspecificidadAsunto(s)
Albuminuria/prevención & control , Bloqueadores del Receptor Tipo 1 de Angiotensina II/administración & dosificación , Presión Sanguínea/efectos de los fármacos , Enfermedades Cardiovasculares/mortalidad , Diabetes Mellitus Tipo 2/tratamiento farmacológico , Imidazoles/administración & dosificación , Tetrazoles/administración & dosificación , HumanosRESUMEN
Cardiovascular involvement is a leading cause of mortality and morbidity in patients with inherited hemoglobinopathies, but it has not been adequately assessed in sickle beta-thalassemia. We evaluated 115 sickle beta-thalassemia patients, aged 34 +/- 14 years, along with 50 healthy controls, by resting echocardiography. Patients with systolic left ventricular (LV) dysfunction or severe pulmonary hypertension (PHT) also underwent left and right cardiac catheterization and cardiac magnetic resonance imaging (CMR). Left and right chamber dimensions, LV mass, and cardiac index were significantly higher in patients compared to controls (p < 0.001 in most cases). Three patients (2.9%) had reduced LV ejection fraction (<55%); mean LV ejection fraction was significantly lower in patients (p < 0.001). Left and right ventricular systolic tissue Doppler indices and LV diastolic tissue Doppler indices were also impaired in patients. All three patients with systolic LV dysfunction had normal coronary arteries and mild myocardial iron load (CMR T2* values, 18-25 ms). Systolic pulmonary artery pressure was significantly higher in patients compared to controls (p = 0.002); PHT was present in 28 patients (27%), while severe PHT in three (2.9%). In three patients with severe PHT, only one had impaired LV ejection fraction and increased pulmonary wedge pressure. Overall, three patients (2.9%) had a history of heart failure, two with systolic LV dysfunction, and one with severe PHT. Cardiac involvement in sickle beta-thalassemia concerns biventricular dilatation and dysfunction along with PHT, leading to congestive heart failure.
Asunto(s)
Cardiopatías/complicaciones , Cardiopatías/fisiopatología , Talasemia beta/complicaciones , Adolescente , Adulto , Anciano , Electrocardiografía , Femenino , Cardiopatías/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Talasemia beta/fisiopatologíaRESUMEN
An acquired diffuse elastic tissue defect that resembles inherited pseudoxanthoma elasticum (PXE) has been noticed with a significant age-related frequency in hemoglobin disorders, especially beta-thalassemia and has been held responsible for a number of complications observed in these cases, some of which are quite severe. We report here two patients with beta-thalassemia intermedia, who presented with severe visual acuity impairment associated with angioid streaks, the typical ocular manifestation of PXE.
Asunto(s)
Estrías Angioides/etiología , Ceguera/etiología , Talasemia beta/complicaciones , Adulto , Estrías Angioides/patología , Ceguera/patología , Femenino , Humanos , Masculino , Persona de Mediana EdadAsunto(s)
Hemoglobinopatías/complicaciones , Hipertensión Pulmonar/etiología , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/tratamiento farmacológico , Hemoglobinopatías/tratamiento farmacológico , Humanos , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/epidemiología , Prevalencia , Talasemia beta/complicaciones , Talasemia beta/tratamiento farmacológicoRESUMEN
BACKGROUND: Homozygous sickle-cell anemia and beta-thalassemia are characterized by impaired endothelial function, while data on arterial stiffness have hitherto been conflicting. We sought to investigate aortic elastic properties and endothelial function in sickle-thalassemia, which combines molecular and clinical features of the above conditions. METHODS AND RESULTS: Forty-seven sickle-thalassemia patients, younger than 45 years, with preserved left ventricular (LV) function and no history of smoking, systemic or pulmonary hypertension, diabetes mellitus, dyslipidemia or thyroid disease, along with 40 healthy controls were studied. Aortic strain, distensibility and stiffness index were calculated by echocardiographically-obtained aortic root diameters. Brachial artery endothelial function was assessed by ultrasonographic evaluation of flow-mediated dilatation (FMD) and nitrate-mediated dilatation (NMD). Left ventricle was assessed by echocardiography. Patients had an impaired FMD (4.2+/-2.9% versus 9.2+/-3.8% in controls, p<0.001) with a preserved NMD (16.9+/-5.6% versus 15.2+/-4.8% in controls, p>0.05). Aortic strain and distensibility were lower and aortic stiffness index was higher in patients compared to controls (8.1+/-4.6 versus 5.8+/-2.9, p<0.01). Indexed LV diameters and mass were higher in patients. Systolic LV function was preserved, while 14.9% of patients had an impaired relaxation transmitral inflow pattern. Patients' LV mass index and diastolic mitral E wave deceleration time were positively correlated with aortic stiffness index (p<0.001). CONCLUSION: Sickle-thalassemia is characterized by a complex vasculopathy, consisting of endothelial dysfunction and increased arterial stiffness, with a global effect on cardiovascular function.
Asunto(s)
Aorta/fisiopatología , Arteria Braquial/fisiopatología , Endotelio Vascular/fisiopatología , Vasodilatación , Talasemia beta/fisiopatología , Adulto , Aorta/diagnóstico por imagen , Arteria Braquial/diagnóstico por imagen , Estudios de Casos y Controles , Ecocardiografía , Elasticidad , Femenino , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Humanos , Hiperemia/fisiopatología , Masculino , Flujo Pulsátil , Flujo Sanguíneo Regional , Función Ventricular Izquierda , Talasemia beta/diagnóstico por imagenRESUMEN
Heart disease is the leading cause of mortality and one of the main causes of morbidity in beta-thalassemia. The clinical spectrum of the thalassemia syndrome ranges from the severe, transfusion--dependent thalassemia major and the asymptomatic carrier state. Thalassemia intermedia represents a milder form and is usually transfusion-independent. Two main factors determine cardiac disease in this form. One is the high output state that results from chronic tissue hypoxia and from hypoxia-induced compensatory reactions. The other is the vascular involvement that leads to an increased pulmonary vascular resistance and an increased systemic vascular stiffness. Valvular abnormalities and iron overload also contribute to a less extent. As a result, both right and left ventricles have to maintain a high cardiac output level through a stiff vascular bed. Right heart involvement with age-related pulmonary hypertension followed by congestive heart failure dominates the clinical picture. Although the left heart is also affected, systolic left ventricular function is usually preserved but this may also be decompensated under conditions characterized by excessive cardiac work load.
Asunto(s)
Cardiopatías/etiología , Talasemia beta/complicaciones , Gasto Cardíaco , Hipoxia de la Célula , Tejido Elástico/patología , Endotelio Vascular/patología , Eritropoyesis , Hemoglobina Fetal/metabolismo , Cardiopatías/fisiopatología , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/fisiopatología , Enfermedades de las Válvulas Cardíacas/etiología , Enfermedades de las Válvulas Cardíacas/fisiopatología , Humanos , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/fisiopatología , Hipoxia/etiología , Absorción Intestinal , Hierro/metabolismo , Sobrecarga de Hierro/etiología , Sobrecarga de Hierro/fisiopatología , Hierro de la Dieta/farmacocinética , Óxido Nítrico/metabolismo , Trombofilia/etiología , Trombofilia/fisiopatología , Vasodilatación , Función Ventricular Izquierda , Talasemia beta/fisiopatologíaRESUMEN
BACKGROUND: Plasma B-type natriuretic peptide (BNP) concentration has significant diagnostic accuracy and prognostic value in various forms of heart disease. Whether BNP is also useful in the evaluation and management of thalassaemia heart disease remains to be determined. METHODS AND RESULTS: Eighty three thalassaemia major patients; 8 with acutely decompensated heart failure (New York Heart Association [NYHA] class III or IV, group A), 25 with NYHA class II symptoms and impaired systolic left ventricular function (ejection fraction<55% or fractional shortening<30%, group B) and 50 with normal systolic function (group C), as well as 50 healthy controls, were studied. Assessment included history, physical examination, Doppler echocardiography and plasma BNP determination. Mean BNP levels were 431+/-219 pg/mL (range, 283-890 pg/mL) in group A, 158+/-31 pg/mL in group B, 176+/-54 pg/mL in group C and 43+/-24 pg/mL in controls. BNP levels were significantly higher in group A (p<0.001), but did not differ between groups B and C. Moreover, BNP was not correlated with left ventricular end-diastolic diameter, left ventricular mass, right ventricular diameter index, Doppler diastolic indexes (except in group C), the mean 2-year serum ferritin concentration or the peak serum ferritin concentration in any of the three patient groups. CONCLUSION: A potential deficiency of BNP-related neurohormonal mechanisms may impair its clinical usefulness in thalassaemia major.
Asunto(s)
Péptido Natriurético Encefálico/sangre , Talasemia beta/sangre , Enfermedad Aguda , Adulto , Análisis de Varianza , Biomarcadores/sangre , Estudios de Casos y Controles , Ecocardiografía Doppler , Femenino , Ferritinas/sangre , Grecia , Insuficiencia Cardíaca/sangre , Insuficiencia Cardíaca/diagnóstico por imagen , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/fisiopatología , Humanos , Masculino , Proyectos de Investigación , Volumen Sistólico , Disfunción Ventricular Izquierda/sangre , Disfunción Ventricular Izquierda/diagnóstico por imagen , Disfunción Ventricular Izquierda/etiología , Disfunción Ventricular Izquierda/fisiopatología , Talasemia beta/complicacionesRESUMEN
Metformin therapy in polycystic ovary syndrome (PCOS) improves metabolic and hormonal profiles. Its therapeutic effect on cardiovascular risk factors is under investigation. Advanced glycation end products (AGEs), well-known atherogenic molecules, were recently found to be elevated in plasma of women with PCOS. The purpose of the study was to investigate the effect of metformin treatment in plasma AGE levels of women with PCOS. This was a descriptive clinical trial. The study involved 22 patients with PCOS (age, 25.09 +/- 1.05 years; body mass index [BMI], 28.44 +/- 1.51 kg/m(2)) and 22 healthy women (age, 26.50 +/- 0.85 years; BMI, 25.62 +/- 1.30 kg/m(2)). Measurements of plasma AGE levels (U/mL) were performed, and the metabolic and hormonal profiles were determined in all subjects. All women with PCOS received a dose of 1700 mg metformin daily for 6 months. AGEs levels were reduced after metformin administration in 22 women with PCOS (9.98 +/- 0.13 [before metformin] vs 9.86 +/- 0.11 [after metformin], P = .05). In a subgroup analysis, of 16 women with PCOS and normal glucose tolerance, the drop of AGE levels was potentiated (9.98 +/- 0.19 [before] vs 9.81 +/- 0.15 [after], P = .02). Body mass index as well as the other parameters studied remained unchanged after metformin therapy apart from a drop of testosterone levels (P = .01) and free androgen index (P = .009). In conclusion, after metformin therapy, the atherogenic AGE molecules were reduced in the serum of women with PCOS . The clinical relevance of this finding in PCOS, a high-risk group for type 2 diabetes mellitus and cardiovascular disease, remains to be seen. Future studies are required to confirm the need of therapeutic intervention for short-term abnormalities and for prevention of long-term sequelae characterizing this syndrome.
Asunto(s)
Productos Finales de Glicación Avanzada/sangre , Hipoglucemiantes/farmacología , Metformina/farmacología , Síndrome del Ovario Poliquístico/tratamiento farmacológico , Adulto , Glucemia/metabolismo , Colesterol/sangre , Femenino , Humanos , Insulina/sangre , Síndrome del Ovario Poliquístico/sangre , Globulina de Unión a Hormona Sexual/metabolismo , Testosterona , Relación Cintura-CaderaRESUMEN
Thalassemia intermedia is a heterogeneous, transfusion-independent form of b-thalassemia, with a clinical course dominated by multi-organ effects of chronic tissue hypoxia, in which hemoglobin F percentage seems to play an important role. We describe the case of a transfusion-independent thalassemia intermedia patient (total hemoglobin 10.7 g/dl) with high hemoglobin F percentage (70%), who presented with persistent leg ulcerations. The patient was successfully treated with one-year exchange blood transfusions, which reduced hemoglobin F percentage to 35%.
Asunto(s)
Recambio Total de Sangre , Úlcera de la Pierna/terapia , Talasemia beta/complicaciones , Adulto , Transfusión Sanguínea , Terapia por Quelación , Deferoxamina/uso terapéutico , Recuento de Eritrocitos , Hemoglobinas/análisis , Humanos , Quelantes del Hierro/uso terapéutico , Úlcera de la Pierna/etiología , Masculino , Talasemia beta/tratamiento farmacológico , Talasemia beta/terapiaRESUMEN
BACKGROUND: Heart disease represents the main determinant of survival in beta-thalassemia, but its particular features in the two clinical forms of the disease, thalassemia major (TM) and thalassemia intermedia (TI), are not completely clarified. METHODS: We compared clinical and echocardiographic global parameters in 131 TM patients who received regular chelation transfusions and were highly compliant with treatment (mean age, 28 +/- 6 years [+/- SD]), and 74 age-matched, TI patients who did not receive chelation transfusions. RESULTS: Congestive heart failure was encountered in five patients with TM (3.8%; age range, 25 to 29 years) and in two patients with TI (2.7%; age range, 37 to 40 years). Systolic left ventricular (LV) dysfunction (ejection fraction < 55% or shortening fraction < 35%) was only encountered in patients with TM (8.4%). Considerable pulmonary hypertension (systolic tricuspid gradient > 35 mm Hg) was only present in TI (23.0%). In the remaining patients without evident heart disease, cardiac dimensions, LV mass, LV shortening and ejection fractions, and cardiac output were significantly higher in patients with TI. LV afterload was higher in patients with TM. LV diastolic early transmitral diastolic peak flow velocity (E)/late transmitral diastolic peak flow velocity (A) ratio was also higher in TM. Systolic and mean pulmonary artery pressures and total pulmonary resistance were higher in both young and old TI patients. CONCLUSION: Regular lifelong transfusion and chelation therapy in TM prevented premature heart disease and pulmonary hypertension, although LV dysfunction still occurred and led to heart failure. The absence of regular therapy in TI, in contrast, preserved systolic LV function but allowed pulmonary hypertension development, which also led to heart failure, starting within the fourth decade of life, a decade later compared to TM.
Asunto(s)
Cardiopatías/etiología , Talasemia beta/complicaciones , Adulto , Gasto Cardíaco , Comorbilidad , Ecocardiografía Doppler , Femenino , Cardiopatías/diagnóstico por imagen , Cardiopatías/epidemiología , Cardiopatías/fisiopatología , Humanos , Hipertensión Pulmonar/epidemiología , Masculino , Función Ventricular Izquierda , Talasemia beta/diagnóstico por imagen , Talasemia beta/epidemiología , Talasemia beta/fisiopatologíaRESUMEN
Cardiac involvement represents the leading cause of mortality in both forms of beta-thalassemia, namely, thalassemia major (TM) and thalassemia intermedia (TI), and pulmonary hypertension (PHT) is part of the cardiopulmonary complications of the disease. PHT was initially documented in a small group of TI patients with right heart failure. In a subsequent study of a large 110-patient series, aged 32.5 +/- 11.4 years, age-related PHT was encountered in nearly 60% of cases, having caused right heart failure in six of them; interestingly, all patients had preserved left ventricular systolic function. Conflicted evidence, however, existed with respect to the development of PHT in heterogeneously treated and young TM populations. To resolve this discrepancy, a recent study compared cardiac disease between two large aged-matched groups of TM (n = 131) and TI (n = 74) patients, both treated uniformly in the currently accepted manner (regular transfusion and chelation therapy in TM, absence of any particular treatment in TI); well-treated TM patients, in contrast to TI patients, did not develop PHT, while systolic left ventricular dysfunction was present only in TM cases. PHT in beta-thalassemia results from a rather complex pathophysiology, in which chronic tissue hypoxia seems to hold a key role. Although both forms of the disease share a common molecular background, the diverse severity of the genetic defect and of the resulting clinical phenotype require a different therapeutic approach. Regular lifelong therapy in TM patients eliminates chronic hypoxia, thereby preventing PHT, whereas the absence of systematic treatment in TI leads to a cascade of reactions that compensate for chronic anemia, but at the same time allow the development of PHT.
Asunto(s)
Hipertensión Pulmonar/etiología , Talasemia beta/complicaciones , Adulto , Factores de Edad , Transfusión Sanguínea , Gasto Cardíaco , Terapia por Quelación , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/fisiopatología , Humanos , Hipertensión Pulmonar/fisiopatología , Hipoxia/etiología , Hipoxia/fisiopatología , Sobrecarga de Hierro/etiología , Sobrecarga de Hierro/fisiopatología , Persona de Mediana Edad , Tromboembolia/etiología , Tromboembolia/fisiopatología , Resistencia Vascular , Disfunción Ventricular Izquierda/etiología , Disfunción Ventricular Izquierda/fisiopatología , Talasemia beta/tratamiento farmacológico , Talasemia beta/fisiopatología , Talasemia beta/terapiaRESUMEN
The association between myelodysplastic syndromes (MDS) and autoimmune manifestations is not uncommon. As a rule, autoimmune abnormalities follow the diagnosis of MDS. We describe here a patient with MDS who developed a striking spectrum of diverse autoimmune disorders, including dermatitis, polyarthritis, and vasculitis, which preceded the clinical appearance of MDS.
Asunto(s)
Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/terapia , Síndromes Mielodisplásicos/diagnóstico , Síndromes Mielodisplásicos/terapia , Anciano , Anciano de 80 o más Años , Artritis/complicaciones , Artritis/diagnóstico , Enfermedades Autoinmunes/complicaciones , Transfusión Sanguínea/métodos , Terapia Combinada , Dermatitis/complicaciones , Dermatitis/diagnóstico , Quimioterapia Combinada , Epoetina alfa , Eritropoyetina/uso terapéutico , Filgrastim , Estudios de Seguimiento , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Humanos , Masculino , Metilprednisolona/uso terapéutico , Síndromes Mielodisplásicos/complicaciones , Proteínas Recombinantes , Medición de Riesgo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Vasculitis/complicaciones , Vasculitis/diagnósticoRESUMEN
Cardiovascular abnormalities are often apparent in sickle cell disease, as in other chronic anaemias. Tissue oxygen extraction and cardiac output are increased, while the activated endothelium along with the sickling process and the vaso-occlusion of the small blood vessels strain further the cardiovascular system. Evidence for a specific myocardial lesion attributable to the sickle cell disease is ambiguous through different studies. This article attempts to review the literature about cardiac disease in sickle cell anaemia.