RESUMEN
A 66-year-old man presented with hemolytic anemia, which required frequent blood transfusion, 6 months after surgical repair of an ascending aortic pseudoaneurysm. Hemolysis was attributed to luminal stenosis caused by graft kinking by laboratory test, CT and four-dimensional magnetic resonance imaging. First, an Excluder cuff was placed at the stenotic site under rapid pacing, but it migrated distally. Thereafter a Palmaz XL stent was placed at the stenotic site, which led to resolution of anemia. In this case, a Palmaz XL stent was successfully used to treat hemolytic anemia caused by graft kinking following ascending aortic surgery.
Asunto(s)
Anemia Hemolítica , Implantación de Prótesis Vascular , Procedimientos Endovasculares , Masculino , Humanos , Anciano , Prótesis Vascular/efectos adversos , Resultado del Tratamiento , Aorta/diagnóstico por imagen , Aorta/cirugía , Stents/efectos adversos , Implantación de Prótesis Vascular/efectos adversos , Anemia Hemolítica/diagnóstico por imagen , Anemia Hemolítica/etiología , Procedimientos Endovasculares/efectos adversosRESUMEN
A 67-year-old male underwent ascending aortic replacement for Stanford type A acute aortic dissection four years ago. Residual false lumen in the aortic root and mild to moderate aortic regurgitation were noted postoperatively. Two and a half years later, he presented with hemolytic anemia and shortness of breath. Computed tomography (CT) revealed aneurysmal aortic root and severely kinked vascular prosthesis, and echocardiography showed severe aortic regurgitation. It was considered that the proximal residual false lumen gradually enlarged, and the graft was pushed up distally and kinked, resulting in hemolytic anemia. Surgical treatment was indicated because of deteriorating shortness of breath due to hemolytic anemia (Hb 6.7 g/dl, LDH 1,528 U/l) and aortic regurgitation. Aortic root replacement was successfully performed and hemolytic anemia improved immediately after surgery.
Asunto(s)
Anemia Hemolítica , Aneurisma de la Aorta Torácica , Disección Aórtica , Insuficiencia de la Válvula Aórtica , Implantación de Prótesis Vascular , Anciano , Anemia Hemolítica/diagnóstico por imagen , Anemia Hemolítica/etiología , Anemia Hemolítica/cirugía , Disección Aórtica/complicaciones , Disección Aórtica/diagnóstico por imagen , Disección Aórtica/cirugía , Aneurisma de la Aorta Torácica/cirugía , Insuficiencia de la Válvula Aórtica/diagnóstico por imagen , Insuficiencia de la Válvula Aórtica/etiología , Insuficiencia de la Válvula Aórtica/cirugía , Prótesis Vascular/efectos adversos , Implantación de Prótesis Vascular/efectos adversos , Disnea , Humanos , Masculino , Resultado del TratamientoRESUMEN
Hereditary spherocytosis (HS) is the most common cause of inherited, nonimmune hemolytic anemia. When inherited in an autosomal dominant fashion, the anemia is typically mild. However, severe, transfusion-dependent anemia is seen in autosomal recessive HS, which is often associated with deficient or absent red blood cell membrane protein alpha-spectrin. We report a 26-year-old para one who was referred to our center at 28 weeks' gestation due to concerns for fetal anemia. Evaluation revealed elevated peak systolic velocity in the middle cerebral artery by Doppler scan and fetal cardiomegaly. Fetal hematocrit obtained by sampling the umbilical vein was 9% confirming severe fetal anemia. Fetal peripheral smear was consistent with hereditary spherocytosis. Genetic analysis of both parents confirmed heterozygosity for the SPTA1 variants (pathogenic variant c.4180del (p.C1394Afs*25), and a variant of uncertain significance, c.1677G>T (p.G449G)) detected by a hemolytic anemia panel in the patient's first child. It is important to consider genetic causes of anemia in patients presenting with severe nonimmune fetal anemia, including autosomal recessive HS. We present a case of autosomal recessive HS with a novel pathogenic variant in the SPTA1 gene which resulted in significant impact on prenatal management.
Asunto(s)
Anemia Hemolítica/genética , Anemia Neonatal/genética , Enfermedades Fetales/genética , Espectrina/genética , Anemia Hemolítica/diagnóstico , Anemia Hemolítica/diagnóstico por imagen , Anemia Hemolítica/patología , Anemia Neonatal/diagnóstico , Anemia Neonatal/diagnóstico por imagen , Anemia Neonatal/patología , Femenino , Enfermedades Fetales/diagnóstico , Enfermedades Fetales/diagnóstico por imagen , Enfermedades Fetales/patología , Humanos , Lactante , Recién Nacido , Masculino , Mutación , Linaje , Embarazo , HermanosRESUMEN
A 66-year-old woman developed marked haemolytic anaemia 2 years after total aortic arch replacement using a branched Dacron graft. Echocardiography confirmed bicuspid aortic valve stenosis. A four-dimensional flow magnetic resonance imaging revealed a jet flow arising from the aortic valve along with the vortex and turbulent flow inside the kinked prosthetic graft at the ascending aorta. She underwent a reoperation to replace the aortic valve and correct the kinking. The estimated energy loss after intervention was relieved to 2.9 mJ/cardiac cycle from 4.3 mJ/cardiac cycle before intervention. The patient's anaemia resolved immediately after the reoperation.
Asunto(s)
Anemia Hemolítica/diagnóstico por imagen , Técnicas de Imagen Cardíaca/métodos , Imagen por Resonancia Magnética/métodos , Complicaciones Posoperatorias/diagnóstico , Anciano , Aorta Torácica/cirugía , Implantación de Prótesis Vascular , Femenino , HumanosRESUMEN
Felt strips are widely used for reinforcement of the aortic stump in surgery for aortic dissection (AD). Postoperative hemolytic anemia (HA) due to an inverted internal felt strip at the aortic stump fixation for AD is extremely rare. A 70-year-old woman underwent ascending aorta replacement for acute type A AD, where both proximal and distal anastomotic sites were reinforced with Teflon felt strips. A week later, macroscopic hematuria and HA emerged. Three-dimensional transesophageal echocardiography (3D-TEE) demonstrated that the proximal inner felt strip turned up and protruded into the aortic inner lumen. At redo surgery, which was performed 2 weeks after the initial surgery, the findings of 3D-TEE were confirmed, and the inverted internal felt strip was replaced with a bovine pericardial strip. The findings of HA disappeared immediately after the second surgery. 3D-TEE is a very informative, valuable modality for accurate diagnosis that leads to a safe surgery.
Asunto(s)
Anemia Hemolítica/diagnóstico por imagen , Aneurisma de la Aorta/cirugía , Disección Aórtica/cirugía , Prótesis Vascular/efectos adversos , Ecocardiografía Tridimensional , Ecocardiografía Transesofágica , Anciano , Anemia Hemolítica/etiología , Disección Aórtica/diagnóstico por imagen , Aneurisma de la Aorta/diagnóstico por imagen , Ecocardiografía Tridimensional/métodos , Ecocardiografía Transesofágica/métodos , Femenino , Humanos , ReoperaciónRESUMEN
INTRODUCTION: Recently defined consensus criteria for the diagnosis of neuromyelitis optica spectrum disorders (NMOSD) allow establishing the diagnosis in patients without elevated AQP4-Ab and optic nerve involvement. According to the new extended definition, NMOSD is closely associated with extensive spinal cord inflammation occurring in the course of systemic autoimmune diseases as sarcoidosis or lupus erythematodes. NMOSD occurring in the course of hematological disease have not yet been reported in the literature. CASE REPORT: A 38 year old male subsequently developed thrombocytopenia, hemolytic anemia and agranulocytosis over a 23 month period. Three months after an episode of agranulocytosis, he noticed ascending sensory disturbances and progressive weakness of his legs. Within two days, symptoms worsened to give almost complete paraplegia and loss of sensation below a midthoracic level. MRI revealed signal hyperintensity and edema in T2-weighted sequences reaching from the 2nd cervical to the 9th thoracic vertebral body. Two years later, he developed a second episode with lesions in the spinal cord and periventricular areas of brain stem and thalamus. CONCLUSION: The relapsing time course and the topographical pattern of central nervous system lesions restricted to axial brain structures and the spinal cord fulfill the criteria that have recently been defined for AQP4-Ab-negative NMO-spectrum disease. Systematic studies on the association of hematological autoimmune phenomena and spinal cord disease are needed to clarify whether this coincidence is just a casual phenomenon or whether it points to a yet undiscovered but perhaps therapeutically interesting link of immunological mechanisms affecting both organ systems.
Asunto(s)
Agranulocitosis/complicaciones , Anemia Hemolítica/complicaciones , Neuromielitis Óptica/complicaciones , Trombocitopenia/complicaciones , Agranulocitosis/diagnóstico por imagen , Agranulocitosis/terapia , Anemia Hemolítica/diagnóstico por imagen , Anemia Hemolítica/terapia , Médula Cervical/diagnóstico por imagen , Humanos , Masculino , Persona de Mediana Edad , Neuromielitis Óptica/diagnóstico por imagen , Neuromielitis Óptica/terapia , Trombocitopenia/diagnóstico por imagen , Trombocitopenia/terapiaAsunto(s)
Lesión Renal Aguda/complicaciones , Anemia Hemolítica/complicaciones , Anemia Hemolítica/cirugía , Defectos del Tabique Interventricular/complicaciones , Defectos del Tabique Interventricular/cirugía , Intervención Coronaria Percutánea , Lesión Renal Aguda/diagnóstico por imagen , Anciano , Anemia Hemolítica/diagnóstico por imagen , Femenino , Defectos del Tabique Interventricular/diagnóstico por imagen , Hemólisis/fisiología , Humanos , UltrasonografíaRESUMEN
OBJECTIVE: Doppler measurement of peak velocity of systolic blood flow in the middle cerebral artery (PVS-MCA) can safely replace invasive testing in the diagnosis of fetal anemia in Rh-alloimmunized pregnancies and PSV-MCA is now the reference technique. However, no study has evaluated its impact in antenatal care and in survival rate. Our objective was to evaluate the impact of the measurement of PVS-MCA in antenatal management and neonatal outcome in maternal red cell alloimmunization requiring in utero transfusion (IUT). STUDY DESIGN: Retrospective study between January 1999 and January 2013. We excluded all cases of hydrops without follow-up before first IUT. From 1999 to 2006, an IUT was indicated on the optical index at 450 nm (Period 1) and was then replaced by the use of PVS-MCA (Period 2). RESULTS: 77 patients were included, 39 in Period 1 (104 IUT) and 38 in Period 2 (89 IUT). 5 cases of hydrops fetalis (12.8%) were diagnosed during the follow up in Period 1 and none during Period 2. The average number of IUT, the delays between 2 IUT and between last IUET and birth were comparable. The total rate of complication per IUT during the first period was 9.6% vs 1.1% during the second one (p=0.01). The overall survival rate in our population was 34/39 (86.8%) during Period 1 vs 38/38 (100%) during Period 2. CONCLUSION: PSV-ACM allowed an improved monitoring with fewer occurrences of hydrops. Conversely, it did not modify antenatal management and timing of delivery.
Asunto(s)
Anemia Hemolítica/diagnóstico por imagen , Transfusión de Sangre Intrauterina , Hidropesía Fetal/diagnóstico por imagen , Arteria Cerebral Media/diagnóstico por imagen , Ultrasonografía Prenatal , Adulto , Amniocentesis , Anemia Hemolítica/inmunología , Anemia Hemolítica/terapia , Peso al Nacer , Velocidad del Flujo Sanguíneo , Transfusión de Sangre Intrauterina/efectos adversos , Femenino , Humanos , Hidropesía Fetal/inmunología , Recién Nacido , Embarazo , Estudios Retrospectivos , Isoinmunización Rh/complicaciones , Tasa de Supervivencia , Ultrasonografía DopplerRESUMEN
We report a rare case of a patient with hemolytic anemia who underwent emergency total arch replacement using the elephant trunk technique for type A acute aortic dissection 3 years earlier. The 78-year-old woman was referred to our hospital for diagnosis of the cause of the hemolytic anemia, which required twice weekly blood transfusion. Although contrast-enhanced computed tomography could not provide sufficient information, transesophageal echocardiography (TEE) revealed graft flection with high velocity. After percutaneous balloon dilatation, the hemolytic anemia improved and follow-up TEE showed no re-stenosis of the dilatation site.
Asunto(s)
Anemia Hemolítica/diagnóstico por imagen , Aorta Torácica/cirugía , Disección Aórtica/terapia , Anciano , Implantación de Prótesis Vascular , Ecocardiografía Transesofágica , Femenino , Humanos , Resultado del TratamientoRESUMEN
Although hemolytic anemia after mitral valve replacement (MVR) is well recognized, hemolytic anemia associated with mitral valve repair is an uncommon condition. Furthermore, persistent and severe hemolytic anemia despite of medical treatment subsequently requiring reoperation is extremely rare. We here report three cases of intractable hemolytic anemia after mitral valve repair leading to MVR. Collision of regurgitant jet into the annuloplasty ring was the speculated mechanism of hemolysis in all cases. After MVR, all of them experienced immediate resolution of the hemolytic anemia.
Asunto(s)
Anemia Hemolítica/diagnóstico por imagen , Anemia Hemolítica/etiología , Prótesis Valvulares Cardíacas/efectos adversos , Insuficiencia de la Válvula Mitral/diagnóstico por imagen , Insuficiencia de la Válvula Mitral/cirugía , Adulto , Anciano , Anemia Hemolítica/cirugía , Femenino , Humanos , Persona de Mediana Edad , Insuficiencia de la Válvula Mitral/complicaciones , Resultado del Tratamiento , UltrasonografíaAsunto(s)
Anemia Hemolítica/diagnóstico , Anemia Hemolítica/etiología , Incompatibilidad de Grupos Sanguíneos/complicaciones , Incompatibilidad de Grupos Sanguíneos/diagnóstico , Hemorragia/diagnóstico , Anciano de 80 o más Años , Anemia Hemolítica/diagnóstico por imagen , Incompatibilidad de Grupos Sanguíneos/diagnóstico por imagen , Diagnóstico Diferencial , Hematoma/diagnóstico , Hematoma/diagnóstico por imagen , Hemorragia/diagnóstico por imagen , Hemorragia/etiología , Humanos , Masculino , Radiografía , Espacio Retroperitoneal/diagnóstico por imagen , Espacio Retroperitoneal/patologíaAsunto(s)
Anemia Hemolítica/diagnóstico por imagen , Eritroblastosis Fetal/diagnóstico por imagen , Fémur/diagnóstico por imagen , Ventrículos Cardíacos/diagnóstico por imagen , Isoinmunización Rh/fisiopatología , Algoritmos , Anemia Hemolítica/epidemiología , Anemia Hemolítica/etiología , Anemia Hemolítica/patología , Biomarcadores , Velocidad del Flujo Sanguíneo , Pesos y Medidas Corporales , Brasil/epidemiología , Eritroblastosis Fetal/epidemiología , Eritroblastosis Fetal/etiología , Eritroblastosis Fetal/patología , Femenino , Fémur/embriología , Fémur/patología , Ventrículos Cardíacos/embriología , Ventrículos Cardíacos/patología , Humanos , Recién Nacido , Masculino , Arteria Cerebral Media/diagnóstico por imagen , Arteria Cerebral Media/fisiopatología , Proyectos Piloto , Embarazo , Prevalencia , Sensibilidad y Especificidad , Índice de Severidad de la Enfermedad , Ultrasonografía PrenatalRESUMEN
Although rare, periprosthetic valvular regurgitation can cause hemolytic anemia. We present the case of a 63-year-old man who had an unusual presentation of hemolytic anemia due to periprosthetic mitral valve regurgitation (PMVR) in the presence of cold agglutinins. Due to high surgical risk, PMVR was percutaneously closed with three Amplatzer devices under the guidance of three-dimensional transesophageal echocardiography.
Asunto(s)
Anemia Hemolítica/etiología , Anemia Hemolítica/prevención & control , Prótesis Valvulares Cardíacas/efectos adversos , Insuficiencia de la Válvula Mitral/etiología , Insuficiencia de la Válvula Mitral/cirugía , Anemia Hemolítica/diagnóstico por imagen , Ecocardiografía Transesofágica/métodos , Humanos , Masculino , Persona de Mediana Edad , Insuficiencia de la Válvula Mitral/diagnóstico por imagen , Cirugía Asistida por Computador/métodos , Resultado del TratamientoRESUMEN
Haemolytic anaemia following mitral valve replacement is uncommon, however in patients who suffer from some degree of perivalvular leak, severe and potentially fatal recurrent intravascular haemolysis can be an annoying problem. We report the cases of two patients with severe haemolytic anaemia observed some years after mitral valve replacement. In one of the two patients the presence of an association between a valvular leak after mitral valve replacement and a calcific atrial wall produced severe and recurrent haemolysis that required multiple blood transfusions. In the second patient the presence of a single valvular leak after mitral valve replacement induced an episode of haemolytic anaemia some years after the operation. These cases point out that in case of unexplained worsening anaemia, a transthoracic (TT) and transesophageal (TE) echocardiogram should be performed, and the possibility of atrial wall alterations in the producing of anaemia should be kept in consideration. In these cases reoperation resolved the recurrence of anemization.
Asunto(s)
Anemia Hemolítica/cirugía , Hemólisis , Válvula Mitral/metabolismo , Anciano , Anemia Hemolítica/diagnóstico por imagen , Anemia Hemolítica/etiología , Calcinosis/complicaciones , Calcinosis/diagnóstico por imagen , Calcinosis/patología , Calcinosis/cirugía , Femenino , Atrios Cardíacos/diagnóstico por imagen , Atrios Cardíacos/patología , Humanos , Masculino , Válvula Mitral/diagnóstico por imagen , Factores de Tiempo , UltrasonografíaRESUMEN
The use of (51)Cr-labeled red blood cells to determine erythrocyte survival in circulation is not a routine procedure. Data are acquired by frequent counting of small-volume blood samples to determine the rate of red cell survival over time. Results are entirely quantitative and can involve semilogarithmic graphing techniques. An accurate study can positively identify hemolytic anemia, a disorder characterized by premature destruction of erythrocytes. Additionally, anatomic sites of premature hemolysis will demonstrate a higher radioactive count than the background organ. This procedure review presents a dual survival and sequestration study positive for hemolytic anemia and hypersplenism in an 83-y-old man with systemic lupus erythematosus. (51)Cr red cell survival and splenic sequestration studies may be considered when clinical history and laboratory studies cannot establish a definitive diagnosis.
Asunto(s)
Anemia Hemolítica/diagnóstico por imagen , Radioisótopos de Cromo , Eritrocitos/diagnóstico por imagen , Hiperesplenismo/diagnóstico por imagen , Lupus Eritematoso Sistémico/diagnóstico por imagen , Anciano de 80 o más Años , Anemia Hemolítica/sangre , Humanos , Hiperesplenismo/sangre , Lupus Eritematoso Sistémico/sangre , Masculino , Cintigrafía , RadiofármacosRESUMEN
OBJECTIVE: To test a new noninvasive ultrasound method for diagnosing fetal anemia in red blood cell isoimmunized pregnancies. METHODS: A diagnostic accuracy study was carried out to determine the cutoff point of an ultrasound measurement, the cardiofemoral index (CFI), calculated using the biventricular outer dimension (BVOD) and femur length to diagnosis severe anemia. The CFI measurement was performed before each of the 336 cordocenteses on 131 fetuses. Diagnosis test analysis and receiver-operating characteristics (ROC) curves were used and the area under the curve (AUC) was calculated to compare the overall accuracy of the CFI for anemia diagnosis, between fetuses with or without previous intrauterine transfusions (IUT). RESULTS: At first cordocentesis (n=131) the AUC was 0.75 (95% CI, 0.66-0.84). For cases where fetuses had undergone 1 previous transfusion (n=88) the AUC was 0.76 (95% CI, 0.64-0.88) and at the time of the third cordocentesis for IUT (n=53) it was 0.73 (95% CI, 0.59-0.86). For a 0.59 CFI threshold to diagnosis fetuses with hemoglobin deficit above 5 g/dL, sensitivity values were 87.2%, 88.0%, and 94.1% respectively for fetuses without IUT, with 1 IUT, and with 2 IUTs. Likelihood ratios for positive (LR+) and negative (LR-) test results were 1.98, 2.05, 1.69 and 0.23, 0.21, 0.13 respectively. CONCLUSION: The cardiofemoral index may be an effective noninvasive marker of severe fetal anemia in high-risk fetuses, with accuracy similar for fetuses either with or without previous transfusions.