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2.
J Cell Mol Med ; 26(9): 2520-2528, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-35355397

RESUMEN

Although numerous patient-specific co-factors have been shown to be associated with worse outcomes in COVID-19, the prognostic value of thalassaemic syndromes in COVID-19 patients remains poorly understood. We studied the outcomes of 137 COVID-19 patients with a history of transfusion-dependent thalassaemia (TDT) and transfusion independent thalassaemia (TIT) extracted from a large international cohort and compared them with the outcomes from a matched cohort of COVID-19 patients with no history of thalassaemia. The mean age of thalassaemia patients included in our study was 41 ± 16 years (48.9% male). Almost 81% of these patients suffered from TDT requiring blood transfusions on a regular basis. 38.7% of patients were blood group O. Cardiac iron overload was documented in 6.8% of study patients, whereas liver iron overload was documented in 35% of study patients. 40% of thalassaemia patients had a history of splenectomy. 27.7% of study patients required hospitalization due to COVID-19 infection. Amongst the hospitalized patients, one patient died (0.7%) and one patient required intubation. Continuous positive airway pressure (CPAP) was required in almost 5% of study patients. After adjustment for age-, sex- and other known risk factors (cardiac disease, kidney disease and pulmonary disease), the rate of in-hospital complications (supplemental oxygen use, admission to an intensive care unit for CPAP therapy or intubation) and all-cause mortality was significantly lower in the thalassaemia group compared to the matched cohort with no history of thalassaemia. Amongst thalassaemia patients in general, the TIT group exhibited a higher rate of hospitalization compared to the TDT group (p = 0.001). In addition, the rate of complications such as acute kidney injury and need for supplemental oxygen was significantly higher in the TIT group compared to the TDT group. In the multivariable logistic regression analysis, age and history of heart or kidney disease were all found to be independent risk factors for increased in-hospital, all-cause mortality, whereas the presence of thalassaemia (either TDT or TIT) was found to be independently associated with reduced all-cause mortality. The presence of thalassaemia in COVID-19 patients was independently associated with lower in-hospital, all-cause mortality and few in-hospital complications in our study. The pathophysiology of this is unclear and needs to be studied in vitro and in animal models.


Asunto(s)
COVID-19 , Sobrecarga de Hierro , Talasemia , COVID-19/complicaciones , Femenino , Hospitales , Humanos , Sobrecarga de Hierro/etiología , Masculino , Oxígeno , Sistema de Registros , Talasemia/complicaciones , Talasemia/terapia
3.
Am J Hematol ; 97(2): E75-E78, 2022 02 01.
Artículo en Inglés | MEDLINE | ID: mdl-34861054
4.
Magn Reson Med ; 73(5): 2030-7, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-25820253

RESUMEN

PURPOSE: A preliminary assessment of the MRI-compatibility of metallic object possibly embedded within the patient is required before conducting the MRI examination. The Magnetic Iron Detector (MID) is a highly sensitive susceptometer that uses a weak magnetic field to measure iron overload in the liver. MID might be used to perform a screening procedure for MRI by determining the ferromagnetic/conductive properties of embedded metallic objects. METHODS: The study was composed by: (i) definition of MID sensitivity threshold; (ii) application of MID in a procedure to characterize the ferromagnetic/conductive properties of metallic foreign objects in 958 patients scheduled for MID examination. RESULTS: The detection threshold for ferromagnetic objects was found to be the equivalent of a piece of wire of length 2 mm and gauge 0.8 mm(2) and, representing purely conductive objects, an aluminum sheet of area 2 × 2 cm(2) . Of 958 patients, 165 had foreign bodies of unknown nature. MID was able to detect those with ferromagnetic and/or conducting properties based on fluctuations in the magnetic and eddy current signals versus control. CONCLUSION: The high sensitivity of MID makes it suitable for assessing the ferromagnetic/conductive properties of metallic foreign objects embedded within the body of patients scheduled for MRI.


Asunto(s)
Cuerpos Extraños/diagnóstico , Hierro , Campos Magnéticos , Imagen por Resonancia Magnética/métodos , Magnetometría/instrumentación , Magnetometría/métodos , Imanes , Tamizaje Masivo , Metales , Prótesis e Implantes , Adulto , Anciano , Contraindicaciones , Femenino , Humanos , Sobrecarga de Hierro/diagnóstico , Masculino , Persona de Mediana Edad , Seguridad del Paciente , Sensibilidad y Especificidad
6.
Haematologica ; 98(4): 555-9, 2013 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23242593

RESUMEN

Several studies have established an association between iron chelation therapy with deferasirox and hematopoietic improvement in patients with myelodysplastic syndromes. There are no data from patients with ß-thalassemia major. In a cross-sectional study, we evaluated the absolute number of several hematopoietic peripheral progenitors (colony-forming unit-granulocyte/macrophage, erythroid burst-forming units, colony-forming unit-granulocyte/erythrocyte/macrophage/megakaryocyte, and long-term culture-initiating cells) in 30 patients with ß-thalassemia major (median age 29.5 years, 40% males) and 12 age-matched controls. For the ß-thalassemia major patients, data on splenectomy status, the type of iron chelator used, and serum ferritin levels reflecting changes in iron status on the chelator were also retrieved. All patients had to be using the same iron chelator for at least 6 months with >80% compliance. The absolute number of all hematopoietic peripheral progenitors was higher in ß-thalassemia major patients than in controls, and varied between splenectomized and non-splenectomized patients (lower number of erythroid burst-forming units and higher numbers of colony-forming unit-granulocyte/macrophage, colony-forming unit-granulocyte/erythrocyte/macrophage/megakaryocyte, and long-term culture-initiating cells). The number of erythroid burst-forming units was significantly higher in patients taking deferasirox (n=10) than in those taking either deferoxamine (n=10) or deferiprone (n=10) (P<0.05). After adjusting for age, sex, splenectomy status, and serum ferritin changes, the association between a higher absolute number of erythroid burst-forming units in deferasirox-treated patients than in patients taking deferoxamine or deferiprone remained statistically significant (P=0.011). In conclusion, in ß-thalassemia major patients, compared with other iron chelators, deferasirox therapy is associated with higher levels of circulating erythroid burst-forming units. This variation is independent of iron status changes and is more likely to be due to the type of chelator.


Asunto(s)
Terapia por Quelación/métodos , Células Madre Hematopoyéticas/efectos de los fármacos , Quelantes del Hierro/uso terapéutico , Talasemia beta/tratamiento farmacológico , Adulto , Benzoatos/uso terapéutico , Recuento de Células Sanguíneas , Ensayo de Unidades Formadoras de Colonias , Estudios Transversales , Deferasirox , Deferiprona , Deferoxamina/uso terapéutico , Células Precursoras Eritroides/citología , Células Precursoras Eritroides/efectos de los fármacos , Femenino , Ferritinas/sangre , Citometría de Flujo , Células Madre Hematopoyéticas/citología , Humanos , Modelos Lineales , Masculino , Análisis Multivariante , Células Progenitoras Mieloides/citología , Células Progenitoras Mieloides/efectos de los fármacos , Piridonas/uso terapéutico , Esplenectomía , Triazoles/uso terapéutico , Adulto Joven , Talasemia beta/sangre
10.
Haematologica ; 96(11): 1708-11, 2011 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-21791473

RESUMEN

Deferiprone was shown to reverse iron deposition in Friedreich's ataxia. This multi-center, unblinded, single-arm pilot study evaluated safety and efficacy of deferiprone for reducing cerebral iron accumulation in neurodegeneration with brain iron accumulation. Four patients with genetically-confirmed pantothenate kinase-associated neurodegeneration, and 2 with parkinsonism and focal dystonia, but inconclusive genetic tests, received 15 mg/kg deferiprone bid. Magnetic resonance imaging and neurological examinations were conducted at baseline, six and 12 months. Chelation treatment caused no apparent hematologic or neurological side effects. Magnetic resonance imaging revealed decreased iron accumulation in the globus pallidus of 2 patients (one with pantothenate kinase-associated neurodegeneration). Clinical rating scales and blinded video rating evaluations documented mild-to-moderate motor improvement in 3 patients (2 with pantothenate kinase-associated neurodegeneration). These results underline the safety and tolerability of deferiprone, and suggest that chelating treatment might be effective in improving neurological manifestations associated with iron accumulation. (Clinicaltrials.gov Identifier: NTC00907283).


Asunto(s)
Quelantes del Hierro/administración & dosificación , Trastornos del Metabolismo del Hierro/tratamiento farmacológico , Hierro/metabolismo , Enfermedades Neurodegenerativas/tratamiento farmacológico , Piridonas/administración & dosificación , Adulto , Anciano , Deferiprona , Femenino , Humanos , Quelantes del Hierro/efectos adversos , Trastornos del Metabolismo del Hierro/complicaciones , Trastornos del Metabolismo del Hierro/diagnóstico por imagen , Trastornos del Metabolismo del Hierro/fisiopatología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Enfermedades Neurodegenerativas/complicaciones , Enfermedades Neurodegenerativas/diagnóstico por imagen , Enfermedades Neurodegenerativas/metabolismo , Proyectos Piloto , Piridonas/efectos adversos , Radiografía
11.
Eur J Echocardiogr ; 12(3): 242-6, 2011 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-21278193

RESUMEN

AIMS: To determine the clinical management of cardiovascular complications, and the extent of cardiac left ventricular (LV) involvement, in a large cohort of homogenously treated patients with thalassaemia major. METHODS AND RESULTS: Participants were ≥ 16 years of age and diagnosed with thalassaemia major requiring regular blood transfusions since the age of 2. Patient characteristics, clinical and echocardiography data for 524 patients were extracted from Webthal®, an Internet-shared database. Patients were considered to have evidence of cardiovascular disease if at least one cardiovascular drug was recorded in their file. The majority of patients (422 of 524; 80.5%) had not taken any cardiovascular drug. Among those who had angiotensin-converting enzyme-inhibitors were the most commonly used (81 patients) and these were used by significantly more males than females (P < 0.01). Patients in whom cardiovascular drugs were prescribed showed evidence of cardiac structural and/or functional abnormalities, inasmuch as fractional shortening and ejection fraction were significantly lower (31.3 vs. 35% and 54.4 vs. 60.6; both P < 0.001) and LV end-diastolic diameter index was significantly higher (32.9 vs. 31.8; P = 0.004). Interestingly, when compared with patients in whom cardiovascular drug therapy was not deemed necessary, transfusion period was longer in treated patients (26.2 vs. 24.5 years; P= 0.002). CONCLUSION: Approximately 19% of regularly transfused and chelated thalassaemia major patients need cardiovascular drug therapy. This subgroup is characterized by a dilated and mildly hypokinetic left ventricle when compared with the majority of thalassaemia major patients, who do not need any cardioactive drug. These data underscore the importance of careful evaluation of cardiac functional status in patients with thalassaemia major. Moreover, this database may serve as a clinically useful reference grid for echocardiograph values in this patient population.


Asunto(s)
Enfermedades Cardiovasculares/etiología , Enfermedades Cardiovasculares/terapia , Talasemia beta/complicaciones , Adolescente , Adulto , Factores de Edad , Análisis de Varianza , Transfusión Sanguínea/métodos , Transfusión Sanguínea/estadística & datos numéricos , Cardiotónicos/uso terapéutico , Enfermedades Cardiovasculares/diagnóstico por imagen , Enfermedades Cardiovasculares/mortalidad , Estudios de Cohortes , Terapia Combinada , Intervalos de Confianza , Ecocardiografía/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Medición de Riesgo , Índice de Severidad de la Enfermedad , Factores Sexuales , Esplenectomía/métodos , Tasa de Supervivencia , Disfunción Ventricular Izquierda/diagnóstico por imagen , Disfunción Ventricular Izquierda/tratamiento farmacológico , Disfunción Ventricular Izquierda/etiología , Adulto Joven , Talasemia beta/diagnóstico , Talasemia beta/terapia
12.
Semin Musculoskelet Radiol ; 15(3): 269-80, 2011 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-21644200

RESUMEN

This article provides an overview of the current use of diagnostic imaging modalities in the evaluation of a heterogeneous group of disorders causing chronic anemias by impaired blood cell production (inherited bone marrow failure syndromes of childhood, aplastic anemia and myelodysplastic syndromes, ß-thalassemia) or increased blood cell destruction (sickle cell disease). During the course of these disorders, various musculoskeletal abnormalities can be encountered, including marrow hyperplasia, reversion of yellow marrow to red marrow, growth disturbances, and, occasionally, extramedullary hematopoiesis. Diagnostic imaging may help the clinician to identify specific complications related to either the disease (e.g., bone infarction and acute osteomyelitis in sickle cell disease) or transfusion (e.g., iron overload due to increased hemolysis) and iron chelation (e.g., desferrioxamine-related dysplastic bone changes and deferiprone-related degenerative arthritis) treatments. In this field, magnetic resonance imaging plays a pivotal role because of its high tissue contrast that enables early assessment of bone marrow changes before they become apparent on plain films or computed tomography or metabolic changes occur on bone scintigraphy or positron emission tomography scan. Overall, familiarity with the range of radiological appearances in chronic anemias is important to diagnose complications and establish appropriate therapy.


Asunto(s)
Anemia/complicaciones , Enfermedades de la Médula Ósea/complicaciones , Enfermedades de la Médula Ósea/patología , Imagen por Resonancia Magnética/métodos , Enfermedades Musculoesqueléticas/patología , Sistema Musculoesquelético/patología , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/diagnóstico por imagen , Anemia de Células Falciformes/patología , Enfermedades de la Médula Ósea/diagnóstico por imagen , Enfermedad Crónica , Humanos , Enfermedades Musculoesqueléticas/diagnóstico por imagen , Sistema Musculoesquelético/diagnóstico por imagen , Síndromes Mielodisplásicos/complicaciones , Síndromes Mielodisplásicos/diagnóstico por imagen , Síndromes Mielodisplásicos/patología , Tomografía Computarizada por Rayos X/métodos , Talasemia beta/complicaciones , Talasemia beta/diagnóstico por imagen , Talasemia beta/patología
13.
J Clin Med ; 9(5)2020 May 25.
Artículo en Inglés | MEDLINE | ID: mdl-32466239

RESUMEN

Sickle-cell disease (SCD) is a worldwide distributed hemoglobinopathy, characterized by hemolytic anemia associated with vaso-occlusive events. These result in acute and chronic multiorgan damage. Bone is early involved, leading to long-term disability, chronic pain and fractures. Here, we carried out a retrospective study to evaluate sickle bone disease (SBD) in a cohort of adults with SCD. We assessed bone density, metabolism and turnover. We also evaluated the presence of fractures and the correlation between SCD severity and skeletal manifestations. A total of 71 patients with SCD were analyzed. The mean age of population was 39 ± 10 years, 56% of which were females. We found osteoporosis in a range between 7% and 18% with a high incidence of vertebral fractures. LDH and AST were predictive for the severity of vertebral fractures, while bone density was not. Noteworthy, we identified -1.4 Standard Deviations T-score as the cutoff for detecting the presence of fractures in patients with SCD. Collectively our data allowed us to develop an algorithm for the management of SBD, which may be useful in daily clinical practice to early intersect and treat SBD.

15.
Intern Emerg Med ; 14(7): 1051-1064, 2019 10.
Artículo en Inglés | MEDLINE | ID: mdl-31385153

RESUMEN

Sickle cell disease (SCD) is the most important hemoglobinopathy worldwide in terms of frequency and social impact, recently recognized as a global public health problem by the World Health Organization. It is a monogenic but multisystem disorder with high morbidity and mortality. Vaso-occlusion, hemolytic anemia and vasculopathy are the hallmarks of SCD pathophysiology. This review focuses both on "time-dependent" acute clinical manifestations of SCD and chronic complications commonly described in adults with SCD. The review covers a broad spectrum of topics concerning current management of SCD targeted at the internists and emergency specialists who are increasingly involved in the care of acute and chronic complications of SCD patients.


Asunto(s)
Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/fisiopatología , Anemia de Células Falciformes/terapia , Manejo de la Enfermedad , Humanos , Medicina Interna/métodos , Medicina Interna/tendencias
16.
Mov Disord ; 23(6): 904-7, 2008 Apr 30.
Artículo en Inglés | MEDLINE | ID: mdl-18383118

RESUMEN

We report the results of iron chelating treatment with deferiprone in a 61-year-old woman with signs and symptoms of neurodegeneration with brain iron accumulation (NBIA). After 6 months of therapy the patient's gait had improved and a reduction in the incidence of choreic dyskinesias was observed. Her gait returned to normal after an additional 2 months of therapy, at which time there was a further reduction in involuntary movements and a partial resolution of the blepharospasm.


Asunto(s)
Encéfalo/metabolismo , Discinesias/patología , Quelantes del Hierro/uso terapéutico , Hierro/metabolismo , Degeneración Nerviosa/patología , Anciano de 80 o más Años , Encéfalo/patología , Discinesias/tratamiento farmacológico , Femenino , Humanos , Imagen por Resonancia Magnética , Degeneración Nerviosa/tratamiento farmacológico , Resultado del Tratamiento
19.
Oncol Rep ; 14(4): 933-40, 2005 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-16142354

RESUMEN

The IEV schedule consisted of epirubicin 100 mg/m2 on day 1, etoposide 150 mg/m2 on days 1-3, and ifosfamide 2.5 g/m2 on days 1-3. Patients who proceeded to haematopoietic stem cell transplants (HDTs) received conditioning therapy with BEAM [for the Hodgkin's Lymphoma (HL) and non-Hodgkin's Lymphoma (NHL) groups], or melphalan 100 mg/m2 and mitoxantrone [for the multiple myeloma (MM) patients]. The study consisted of 65 patients with a median age of 53 years: 27 had aggressive NHL, 20 had HL, 7 had indolent NHL, and 11 had MM. Fifty-five patients received IEV for a disease that was refractory to conventional induction regimens, or that was in first or second relapse; 4 patients were treated with IEV while in complete response (CR) after chemotherapy in order to mobilise peripheral blood stem cells (PBSCs). Ninety percent of patients with HL responded to IEV, and 85% achieved CR. Both aggressive and indolent NHLs were less responsive (ORR 50 and 33%, respectively; CRR 41 and 16.5%, respectively). MM patients displayed an intermediate responsiveness (ORR 50% and CRR 30%). IEV was well tolerated in most patients. No life- threatening infections were recorded. PBSC mobilisation was successful in 37 out of 39 patients (95%) and led to the collection of a median of 16, 12, and 13.7 x 10(6) CD34+ cells/kg in patients with HL, NHL, and MM, respectively. All 37 patients underwent an autologous stem cell transplant following a 1 to 2 month interval after the end of IEV. Two patients were submitted to an allogeneic transplant. The median overall survival rate in HL, aggressive NHL, and indolent NHL is 32 (5-60), 16 (2-46), and 14 (4-42) months, respectively. Median EFS is 31 (5-60), 7 (2-46), and 7.5 (4-42) months, respectively. In conclusion, our study confirms that IEV +/- HDT is a well-tolerated and effective salvage treatment for lymphoid malignancies, and that IEV acts as an excellent stem cell mobiliser.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Epirrubicina/administración & dosificación , Etopósido/administración & dosificación , Enfermedad de Hodgkin/tratamiento farmacológico , Ifosfamida/administración & dosificación , Linfoma no Hodgkin/tratamiento farmacológico , Mieloma Múltiple/tratamiento farmacológico , Trasplante de Células Madre de Sangre Periférica/métodos , Terapia Recuperativa/métodos , Adulto , Anciano , Anciano de 80 o más Años , Antígenos CD34/biosíntesis , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Carmustina/administración & dosificación , Citarabina/administración & dosificación , Femenino , Humanos , Masculino , Melfalán/administración & dosificación , Persona de Mediana Edad , Mitoxantrona/administración & dosificación , Oportunidad Relativa , Podofilotoxina/administración & dosificación , Recurrencia , Inducción de Remisión , Factores de Tiempo , Acondicionamiento Pretrasplante/métodos , Resultado del Tratamiento
20.
Clin Case Rep ; 3(11): 952-4, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-26576280

RESUMEN

Patients with transfusion-dependent myelodysplastic syndromes (MDS) have an increased risk of cardiac events, due to both chronic anemia and iron overload. Here, we report the recovery of cardiac function after an intensive iron chelation therapy in a MDS patient who had developed heart failure due to iron overload.

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