Ruptura atraumática del bazo como forma de presentación de la una amilosis AL en un paciente joven / Spontaneous non-traumatic splenic rupture as a presentation form of light chain amyloidosis. Report of one case

We report a 35-year-old sportive man who was admitted to the emergency department for worsening of acute spontaneous abdominal pain appearing at rest. He only referred having lifted a tree trunk the day before, but he was used to perform such physical efforts. The clinical course at the emergency department was marked by the development of severe anemia secondary to a progressive splenic hematoma and acute pulmonary distress. The patient benefited from total splenectomy. Laboratory data showed hypogammaglobulinemia, proteinuria and the anatomopathological examinations of both spleen and kidneys were consistent with light chain amyloidosis.

Renal Amyloidosis Secondary to ANCA-Associated Vasculitis: A Case Report / 中国医学科学杂志(英文版)

Renal amyloidosis secondary to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare. Here, we reported a 77-year-old woman with ANCA-associated vasculitis. Renal biopsy with Masson trichrome staining showed pauci-immune crescentic glomerulonephritis, and electron microscopy showed amyloid deposition in the mesangial area. Immunofluorescence revealed kappa light chain and lambda light chain negative. Bone marrow biopsy revealed no clonal plasma cell. Finally, she was diagnosed as ANCA-associated vasculitis with secondary renal amyloid A amyloidosis.

Systemic amyloidosis manifestation in a patient with psoriatic arthritis

Abstract Systemic amyloidosis secondary to psoriatic arthritis is rare, and published data are based mainly on case reports and are associated with increased mortality. This is the report of a patient with long-term psoriatic arthritis and chronic sialadenitis, who showed an inadequate response to therapy. The diagnosis of secondary amyloidosis was attained through biopsies of genital skin lesions. Although very rare, it is important that dermatologists and general practitioners consider the possibility of amyloidosis in patients with chronic inflammatory diseases, since an early intervention can be implemented, and thus, the prognosis of this condition can be improved.

Amiloidosis cardiaca secundaria a macroglobulinemia de Waldenström / Cardiac amyloidosis secondary to Waldenström macroglobulinemia

Introducción: La macroglobulinemia de Waldenström constituye una neoplasia hematológica del grupo de las gammapatías monoclonales, que incluye síntomas sistémicos y relacionados al incremento de la paraproteína M. Objetivo: Describir un caso de amiloidosis cardiaca asociada a macroglobulinemia. Caso clínico: Paciente masculino que fue admitido por astenia, disfonía, y durante su evolución desarrolló disnea progresiva, insuficiencia cardiaca y efusión pleural. Adicionalmente, la ecocardiografía mostró patrón granular miocárdico, y la biopsia pleural resultó positiva para la tinción rojo congo. Posteriormente, recibió tratamiento con bortezomib, dexametasona y rituximab con evolución favorable. Conclusiones: En esta enfermedad el diagnóstico temprano es una ventaja importante para la supervivencia. Es por esa razón, que su manejo es paliativo de las manifestaciones cardiacas. El presente caso pone en manifiesto un reto diagnóstico, en el cual se deben tomar en cuenta las etiologías menos frecuentes de insuficiencia cardiaca(AU)

Introduction: Waldenström's macroglobulinemia is a hematological neoplasm belonging to the group of monoclonal gammopathies, which includes systemic symptoms and those related to an increase in M paraprotein. Objective: To describe a case of cardiac amyloidosis associated with macroglobulinemia. Clinical case: Male patient who was admitted for asthenia, dysphonia, and who, during his evolution, developed progressive dyspnea, heart failure and pleural effusion. Additionally, echocardiography showed myocardial granular pattern, while pleural biopsy was positive for Congo red staining. Subsequently, he received treatment with bortezomib, dexamethasone and rituximab, with favorable evolution. Conclusions: In this disease, early diagnosis is an important advantage for survival. Therefore, its management is palliative of cardiac manifestations. The present case shows a diagnostic challenge, in which the less frequent etiologies of heart failure must be taken into account(AU)

Amiloidosis AL y mieloma múltiple: macroglosia como primera manifestación de una combinación infrecuente / AL amyloidosis and multiple myeloma: macroglossia as first manifestation of a rare combination

El diagnóstico diferencial de la macroglosia es amplio y puede estar en el contexto de una patología localizada o una enfermedad sistémica. Dentro de las enfermedades sistémicas que se manifiestan con macroglosia se describe la amiloidosis, caracterizada por un depósito irreversible de una proteína amorfa y fibrilar El compromiso de esta patología en el territorio de cabeza y cuello es infrecuente y su depósito en la lengua corresponde a menos del 9% de los casos. Se presenta el caso de un paciente que consultó por dolor en hemilengua derecha, disfagia y baja de peso. Se estudió inicialmente con exámenes de laboratorio y nasofibroscopía, sin hallazgos concluyentes. Dado persistencia de síntomas se realizó resonancia nuclear magnética (RM) que mostraba signos sugerentes de enfermedad de depósito, confirmándose mediante estudios histopatológicos una amiloidosis sistémica secundaria. El paciente fue derivado a hematología para completar estudio e iniciar el tratamiento. La amiloidosis sistémica es un diagnóstico infrecuente, que debe ser considerado por su mal pronóstico vital. El diagnóstico en etapas iniciales puede mejorar sustancialmente la sobrevida y calidad de vida de aquellos que padecen la enfermedad. Por lo anterior, es necesario completar un estudio acabado de la patología, apoyándose en métodos no invasivos como la RM.

The differential diagnosis of macroglossia is broad, and it may be present in the context of a localized pathology or a systemic disease. One of the systemic diseases that present macroglossia is amyloidosis, which is characterized by an irreversible deposit of an amorphous and fibrillar protein. The manifestation of this pathology in head and neck territory is infrequent, and its deposit in the tongue represents less than 9% of all types of amyloidosis. We present the case of a patient, who consulted with pain in the tongue, dysphagia, and weight loss. He was initially studied with laboratory tests and a nasofibroscopy with no conclusive findings. Given the persistence of symptoms, magnetic resonance imaging (MRI) was performed, showing signs of an infiltrative disease, which was confirmed as secondary systemic amyloidosis through histopathological studies. Given the above, the patient was referred to hematology to start treatment. Systemic amyloidosis is an uncommon diagnosis that should be suspected since it implies a poor vital prognosis. Moreover, an early diagnosis can substantially improve the survival rate and quality of life of those who suffer this disease. Therefore, a comprehensive study of this condition is needed, complementing with non-invasive methods such as MRI.

Amiloidosis cardiaca: estudio de caso y revisión bibliográfica / Cardiac amyloidosis: Case study and bibliographic review

Resumen La amiloidosis es una enfermedad multisistémica, originada por un plegamiento proteico anormal el cual a su vez genera su depósito y acumulación en diferentes tejidos. A nivel cardiovascular, el amiloide se deposita en el tejido miocárdico generando las manifestaciones típicas de la enfermedad. Presenta hallazgos electrocardiográficos y ecocardiográficos distintivos que proporcionan una ayuda invaluable en el diagnóstico de la amiloidosis cardiaca. En el presente artículo se expone un caso de un paciente portador de amiloidosis AL, quien exhibe síntomas de falla cardiaca y en el cual se documenta posteriormente severo compromiso cardiovascular. Además, se presenta una revisión de las manifestaciones cardiovasculares y el diagnóstico de dicha patología.

Abstract Amyloidosis is a multisystemic disease, originated by an abnormal protein folding which in turn generates its deposit and accumulation in different tissues. At a cardiovascular level, amyloid is deposited in the myocardial tissue and thus generating the typical manifestations of the disease. It presents distinctive electrocardiographic and echocardiographic findings that provide invaluable help in the diagnosis of cardiac amyloidosis. In this article, a case of a patient with AL amyloidosis is presented. Said patient developed symptoms of heart failure, which later progressed into severe cardiovascular compromise. Furthermore, a review of the cardiovascular manifestations and the diagnosis of said pathology is presented.

Papel del médico de familia en el diagnóstico concomitante de mieloma y amiloidosis primaria en una misma paciente: caso clínico / Multiple myeloma associated with a secondary amyloidosis: report of one case

Multiple Myeloma is a myeloproliferative disorder of plasma cells, which may be complicated with secondary amyloidosis. We report a 48 year old woman consulting to primary care for weight loss and malaise. An initial laboratory study revealed a hypogammaglobulinemia with a monoclonal component and lambda light chains. These results motivated her derivation to hematology: her serum calcium was 11.8 mg/dl, immunofluorescence showed a monoclonal component of lambda chains and urine Bence-Jones protein was positive. A bone marrow biopsy confirmed plasma cell infiltration. A Congo-red stain of a rectal biopsy was positive. The patient was treated with thalidomide, bortezomid and dexamethasone.

Light chain cardiac amyloidosis - a rare cause of heart failure in a young adult

SUMMARY Cardiac amyloidosis is an infiltrative cardiomyopathy, resulting from amyloid deposition within the myocardium. In primary systemic (AL-type) amyloidosis, the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia, and cardiac involvement occurs in up to 50% of the patients We present a case of a 43-year-old man, with complaints of periodical swollen tongue and xerostomia, bleeding gums and haematuria for two months. His blood results showed normocytic anaemia, thrombocytopenia and a high spontaneous INR, therefore he was referred to the Internal Medicine clinic. In the first visit, he showed signs and symptoms of overt congestive heart failure and was referred to the emergency department. The electrocardiogram showed sinus tachycardia and low voltage criteria. Echocardiography showed biventricular hypertrophy with preserved ejection fraction, restrictive physiology with elevated filling pressures, thickened interatrial septum and atrioventricular valves, small pericardial effusion and relative "apical sparing" on 2D longitudinal strain. Cardiac MRI showed diffuse subendocardial late enhancement. Serum protein electrophoresis was inconclusive, however urine analysis revealed nephrotic range proteinuria, positive Bence Jones protein and an immunofixation test with a monoclonal lambda protein band. Abdominal fat biopsy was negative for Congo red stain, nevertheless a bone marrow biopsy was performed, revealing lambda protein monoclonal plasmocytosis, confirming the diagnosis of primary systemic amyloidosis. This case represents a rare cause of heart failure in a young adult. Low-voltage QRS complexes and typical echocardiography features should raise the suspicion for cardiac amyloidosis. Prognosis is dictated by the level of cardiac involvement; therefore, early diagnosis and treatment are crucial.

RESUMO A amiloidose cardíaca corresponde a uma miocardiopatia infiltrativa, resultante do depósito da proteína amiloide no miocárdio. Na amiloidose sistêmica primária (tipo AL), a proteína amiloide é composta por cadeias leves que resultam de discrasia dos plasmócitos, havendo envolvimento cardíaco em até 50% dos doentes. Apresentamos o caso de um homem de 43 anos, com queixas de edema periódico da língua e xerostomia, hemorragia gengival e hematúria há dois meses. Analiticamente havia a destacar anemia normocítica, trombocitopenia e um INR alto espontâneo, pelo que foi referenciado à consulta de Medicina Interna. Na primeira consulta, apresentou-se com sinais de insuficiência cardíaca congestiva franca, pelo que foi referenciado ao Serviço de Urgência. O eletrocardiograma demonstrou taquicardia sinusal e critérios de baixa voltagem. O ecocardiograma revelou hipertrofia biventricular com fração de ejeção preservada, fisiologia restritiva com elevação das pressões de enchimento, espessamento do septo interauricular e das válvulas auriculoventriculares, derrame pericárdico ligeiro e padrão de apical sparing no strain longitudinal 2D. Realizou ainda ressonância magnética cardíaca, que mostrou realce tardio subendocárdico difuso. A eletroforese das proteínas foi inconclusiva, contudo a análise da urina revelou proteinúria no espectro nefrótico, presença de proteína de Bence Jones e um teste de imunofixação com uma banda monoclonal de cadeias lambda. A biópsia da gordura abdominal foi negativa. Não obstante, foi realizada uma biópsia da medula óssea, verificando-se plasmocitose monoclonal lambda, o que confirmou o diagnóstico de amiloidose primária sistêmica. Este caso representa uma causa rara de insuficiência cardíaca no jovem adulto. A baixa voltagem no eletrocardiograma e os achados ecocardiográficos típicos devem fazer suspeitar de amiloidose cardíaca. O prognóstico é ditado pelo nível de envolvimento cardíaco, motivo pelo qual o diagnóstico e o tratamento precoces são essenciais.

Miastenia gravis y su asociación con trastornos linfoproliferativos: casos clínicos / Myasthenia gravis and its association with lymphoproliferative disorders: a case series

Myasthenia gravis (MG) is a rare autoimmune disease of the neuromuscular junction. It is characterized by variable weakness and excessive fatigability of skeletal muscles. In the last few years, numerous reports have been published showing the association between autoimmune diseases, such as systemic erythematous lupus or rheumatoid arthritis, with lymphoid neoplasias. The association between MG and lymphoid neoplasia seems to be less frequent. To analyze this association we reviewed the MG patients in the Department of Neurology, Hospital Salvador of Santiago, Chile. During a three-year period we identified four patients who developed different lymphoproliferative disorders: two with B-cell lymphoma, one with chronic lymphocytic leukaemia and one plasmacytoma with an associated amyloidosis. The MG was generalized but mild, all cases classified as type IIa according to the definition proposed by the MG Foundation of America. The neoplasia appeared two to 36 years after the onset of MG. These cases provide additional evidence of the association between MG and lymphoproliferative disorders.

Clinical outcomes and survival in AA amyloidosis patients / Desfechos clínicos e sobrevida em pacientes com amiloidose AA

Abstract Aim Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. Methods: A total of 81 patients (51 males, 30 females) with renal biopsy proven amyloid A amyloidosis were analyzed retrospectively. The patients were divided into good and poor outcomes groups according to survival results. Results: Most of the patients (55.6%) had nephrotic range proteinuria at diagnosis. Most frequent underlying disorders were familial Mediterranean fever (21.2%) and rheumatoid arthritis (10.6%) in the good outcome group and malignancy (20%) in the poor outcome group. Only diastolic blood pressure in the good outcome group and phosphorus level in the poor outcome group was higher. Serum creatinine levels increased after treatment in both groups, while proteinuria in the good outcome group decreased. Increase in serum creatinine and decrease in estimated glomerular filtration rate of the poor outcome group were more significant in the good outcome group. At the time of diagnosis 18.5% and 27.2% of all patients had advanced chronic kidney disease (stage 4 and 5, respectively). Median duration of renal survival was 65 ± 3.54 months. Among all patients, 27.1% were started dialysis treatment during the follow-up period and 7.4% of all patients underwent kidney transplantation. Higher levels of systolic blood pressure [hazard ratios 1.03, 95% confidence interval: 1-1.06, p = 0.036], serum creatinine (hazard ratios 1.25, 95% confidence interval: 1.07-1.46, p = 0.006) and urinary protein excretion (hazard ratios 1.08, 95% confidence interval: 1.01-1.16, p = 0.027) were predictors of end-stage renal disease. Median survival of patients with organ involvement was 50.3 ± 16 months. Conclusion Our study indicated that familial Mediterranean fever constituted a large proportion of cases and increased number of patients with idiopathic amyloid A amyloidosis. Additionally, it was observed that patient survival was not affected by different etiological causes in amyloid A amyloidosis.

Resumo Objetivo: A amiloidose AA é uma complicação rara de condições inflamatórias crônicas. A maior parte dos pacientes com amiloidose AA apresenta nefropatia, que leva à insuficiência renal e à morte. Estudaram-se as características clínicas e a sobrevida em pacientes com amiloidose AA. Métodos: Analisaram-se retrospectivamente 81 pacientes (51 homens, 30 mulheres) com amiloidose AA comprovada por biópsia renal. Os pacientes foram divididos em grupos de desfecho bom e ruim de acordo com os resultados de sobrevida. Resultados: A maior parte dos pacientes (55,6%) tinha proteinúria na faixa nefrótica no momento do diagnóstico. Os distúrbios subjacentes mais frequentes foram a febre familiar do Mediterrâneo (FFM, 21,2%) e a artrite reumatoide (10,6%) no grupo de desfecho bom e a malignidade (20%) no grupo de desfecho ruim. Somente a pressão arterial diastólica no grupo de desfecho bom e o nível de fósforo no grupo de desfecho ruim foram mais elevados. Os níveis séricos de creatinina aumentaram após o tratamento em ambos os grupos, enquanto a proteinúria diminuiu no grupo de desfecho bom. O aumento na creatinina sérica e a diminuição na TFGe do grupo de desfecho ruim foram mais significativos no grupo de desfecho bom. No momento do diagnóstico, 18,5% e 27,2% de todos os pacientes tinham doença renal crônica avançada (estágios 4 e 5, respectivamente). A duração média da sobrevida renal foi de 65 ± 3,54 meses. Entre todos os pacientes, 27,1% iniciaram tratamento de diálise durante o período de seguimento e 7,4% de todos os pacientes foram submetidos a transplante renal. Níveis elevados de pressão arterial sistólica [taxas de risco (HR) 1,03, intervalo de confiança (IC) de 95%: 1 a 1,06, p = 0,036], creatinina sérica (HR 1,25, IC 95%: 1,07 a 1,46, p = 0,006) e excreção urinária de proteínas (HR 1,08, IC 95%: 1,01 a 1,16, p = 0,027) foram preditores de doença renal terminal. A mediana da sobrevida de pacientes com comprometimento de órgãos foi de 50,3 ± 16 meses. Conclusão: O presente estudo indicou que a FFM constituiu uma grande proporção de casos e crescente quantidade de pacientes com amiloidose AA idiopática. Adicionalmente, observou-se que a sobrevida do paciente não foi afetada pelas diferentes causas etiológicas na amiloidose AA.

Disfunção aguda de cabo-eletrodo atrial secundária a acometimento cardíaco por amiloidose / Acute atrial lead dysfunction secondary to cardiac amyloidosis

A amiloidose cardíaca, uma doença rara, pode cursar com insuficiência cardíaca por infiltração das paredes atrial e ventricular, dificultando também sense e limiares adequados quando do implante de marcapasso. Relatamos o caso de uma paciente que apresentou curso atípico, com rápida infiltração da parede atrial e perda da função ventricular, apesar da apresentação inicial com ecocardiograma e ressonância magnética normais. O caso ilustra o acometimento progressivo da doença de base em diversos aspectos cardíacos, inicialmente no sistema de condução atrioventricular, progredindo para maior fibrose atrial e acometimento miocárdico difuso. Os métodos complementares de ecocardiograma e ressonância magnética aumentaram o grau de suspeição da doença e nos permitiram fazer o diagnóstico específico

Cardiac amyloidosis, a rare disease, may lead to heart failure caused by infiltration of the atrial and ventricular walls, making it difficult to obtain sensed atrial stimulation and adequate thresholds at the time of pacemaker implantation. We report the case of a patient with an atypical outcome, rapid atrial wall infiltration and loss of ventricular function, despite the initial presentation with normal echocardiogram and MRI. This case illustrates the progression of the baseline disease, initially in the atrioventricular conduction system, leading to greater atrial fibrosis and diffuse myocardial involvement. The complementary echocardiogram and magnetic resonance imaging improved the level of suspicion of the disease and enabled us to make a specific diagnosis

Cardiac Amyloidosis and its New Clinical Phenotype: Heart Failure with Preserved Ejection Fraction / Amiloidose Cardíaca e seu Novo Fenótipo Clínico: Insuficiência Cardíaca com Fração de Ejeção Preservada

Abstract Heart failure with preserved ejection fraction (HFpEF) is now an emerging cardiovascular epidemic, being identified as the main phenotype observed in clinical practice. It is more associated with female gender, advanced age and comorbidities such as hypertension, diabetes, obesity and chronic kidney disease. Amyloidosis is a clinical disorder characterized by the deposition of aggregates of insoluble fibrils originating from proteins that exhibit anomalous folding. Recently, pictures of senile amyloidosis have been described in patients with HFpEF, demonstrating the need for clinical cardiologists to investigate this etiology in suspect cases. The clinical suspicion of amyloidosis should be increased in cases of HFPS where the cardio imaging methods are compatible with infiltrative cardiomyopathy. Advances in cardio imaging methods combined with the possibility of performing genetic tests and identification of the type of amyloid material allow the diagnosis to be made. The management of the diagnosed patients can be done in partnership with centers specialized in the study of amyloidosis, which, together with the new technologies, investigate the possibility of organ or bone marrow transplantation and also the involvement of patients in clinical studies that evaluate the action of the new emerging drugs.

Resumo A insuficiência cardíaca com fração de ejeção preservada (ICFEP) é hoje uma epidemia cardiovascular emergente, sendo identificada como o principal fenótipo observado na prática clínica. Está mais associado ao sexo feminino, idade avançada e a comorbidades como hipertensão arterial, diabetes, obesidade e doença renal crônica. A amiloidose é uma desordem clínica caracterizada pelo depósito de agregados de fibrilas insolúveis originadas de proteínas que apresentam dobramento anômalo. Recentemente, têm sido descritos quadros de amiloidose senil em pacientes com ICFEP, demonstrando a necessidade de os cardiologistas clínicos investigarem esta etiologia em casos suspeitos. Deve-se aumentar a suspeição clínica de amiloidose diante dos casos de ICFEP onde os métodos de cardioimagem sejam compatíveis com o quadro de cardiomiopatia infiltrativa. Os avanços nos métodos de cardioimagem aliados à possibilidade de realização de testes genéticos e identificação do tipo do material amiloide permitem a realização do diagnóstico. O manejo dos pacientes diagnosticados pode ser feito em parceria com centros especializados no estudo de amiloidose, que, aliados às novas tecnologias, investigam a possibilidade de transplante de órgãos ou medula óssea e também o envolvimento dos pacientes em estudos clínicos que avaliam a ação das novas drogas emergentes.

Primary Systemic Amyloidosis Involving the Lacrimal Sac

No abstract available.

Familial Mediterranean Fever is associated with abnormal ventricular repolarization indices / La fiebre mediterránea familiar se asocia a anomalías en la repolarizacıón ventricular

Background: Cardiac arrhythmias can be a part of cardiovascular involvement in some rheumatic diseases, but data about familial Mediterranean fever (FMF) are conflicting. Aim: To search for abnormalities in ventricular repolarization indices in FMF patients. Patients and Methods: Seventy seven FMF patients and 30 age/gender comparable healthy controls were included. All patients were attack free and subjects with disease or drugs that are known to alter cardiac electrophysiology were excluded. Electrocardiographic data were obtained and analyzed. Results: Twelve FMF patients had amyloidosis. QT and QTc intervals were within the normal ranges and similar between FMF patients and healthy controls. QT dispersion, peak to end interval of T wave (Tpe), Tpe/QT and Tpe/QTc ratios were significantly higher in FMF patients than in healthy controls. Patients with amyloidosis had significantly higher QT dispersion, Tpe, Tpe/QT and Tpe/QTc than their counterparts without FMF. Levels of proteinuria were moderately correlated with QT dispersion, Tpe, Tpe/QT and Tpe/QTc. Conclusions: FMF patients may have an increased risk for arrhythmias.

Antecedentes: Las arritmias cardíacas pueden ser parte del compromiso cardíaco en enfermedades reumáticas, sin embargo, no se sabe con certeza si esto ocurre en la fiebre mediterránea familiar (FMF). Objetivo: Buscar anomalías en la repolarización ventricular en pacientes con FMF. Pacientes y Métodos: Sesenta y siete pacientes como FMF y 30 controles sanos pareados por edad y género fueron estudiados. Todos los pacientes estaban en período intercrítico y no usaban medicamentos o tenían enfermedades concomitantes que pudieran causar anomalías electrocardiográficas. Se analizaron los electrocardiogramas de estos participantes. Resultados: Veinte pacientes con FMF tenían amiloidosis. Los intervalos QT y QTc eran normales y similares entre pacientes y controles. La dispersión del intervalo QT, el intervalo desde el peak al final de la onda T (Tpe), las razones Tpe/QT y Tpe/QTc fueron significativamente más altos en los pacientes que en los controles. Los pacientes con amiloidosis tenían una dispersión de QT, Tpe, Tpe/QT y Tpe/QTc mayores que sus pares sin la condición. Los niveles de proteinuria se correlacionaron moderadamente con los parámetros antes mencionados. Conclusiones: Los pacientes con FMF tienen mayor riesgo de arritmias.

Resonancia magnética en el diagnóstico de amiloidosis cardíaca / Magnetic resonance imaging in the diagnosis of cardiac amyloidosis

La resonancia magnética cardíaca (RMC) es un método no invasivo que provee información acerca de la anatomía, función y caracterización tisular del miocardio, llegando a ser de gran utilidad en el diagnóstico y diferenciación de trastornos infiltrativos como la amiloidosis. La amiloidosis cardíaca (AC) es una miocardiopatía restrictiva resultado del depósito de amiloide en el corazón, que determina una semiología característica en la RM que permite establecer el diagnóstico en la mayoría de los casos. Los hallazgos por RMC incluyen hipertrofia miocárdica del ventrículo izquierdo (HVI) con realce tardío positivo en el ventrículo izquierdo (VI) y el resto de las cámaras cardíacas, asociado a alteración en la cinética del gadolinio con anulación del pool sanguíneo y hallazgos adicionales, como derrame pleural o pericárdico o ambos, que apoyan el diagnóstico1-3. Presentamos el caso de una paciente con diagnóstico de amiloidosis sometida a RMC en donde se demuestran los hallazgos característicos de esta patología. (AU)

Cardiac magnetic resonance imaging (MRI) is a noninvasive method of image that provides information about the anatomy, function and tissue characterization, becoming very useful in the diagnosis and differentiation of infiltrative disorders such as amyloidosis. Cardiac amyloidosis (CA) is a restrictive cardiomyopathy result of amyloid deposition in the heart. MRI findings include myocardial hypertrophy with positive late gadolinium enhancement (LGE) in the left ventricle (LV) associated with the altered kinetics of gadolinium and additional findings as pleural and pericardial effusion that support the diagnosis. We report the case of a patient diagnosed with amyloidosis showing the characteristic MR findings in this pathology. (AU)