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1.
Transplantation ; 104(8): 1703-1711, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32732850

RESUMO

BACKGROUND: There are limited data on the outcome of transplant recipients with familial Mediterranean fever (FMF)-associated AA amyloidosis. The aim of the present study is to evaluate demographic, clinical, laboratory, and prognostic characteristics and outcome measures of these patients. METHODS: Eighty-one renal transplant recipients with FMF-associated AA amyloidosis (group 1) and propensity score-matched transplant recipients (group 2, n = 81) with nonamyloidosis etiologies were evaluated in this retrospective, multicenter study. Recurrence of AA amyloidosis was diagnosed in 21 patients (group 1a), and their features were compared with 21 propensity score-matched recipients with FMF amyloidosis with no laboratory signs of recurrence (group 1b). RESULTS: The risk of overall allograft loss was higher in group 1 compared with group 2 (25 [30.9%] versus 12 [14.8%]; P = 0.015 [hazard ratio, 2.083; 95% confidence interval, 1.126-3.856]). Patients in group 1 were characterized by an increased risk of mortality compared with group 2 (11 [13.6%] versus 0%; P = 0.001 [hazard ratio, 1.136; 95% confidence interval, 1.058-1.207]). Kaplan-Meier analysis revealed that 5- and 10-year patient survival rates in group 1 (92.5% and 70.4%) were significantly lower than in group 2 (100% and 100%; P = 0.026 and P = 0.023, respectively). Although not reaching significance, overall, 5- and 10-year graft survival rates (57.1%, 94.7%, and 53.8%, respectively) in group 1a were worse than in group 1b (76.2%, 95%, and 77.8%, respectively; P = 0.19, P = 0.95, and P = 0.27, respectively). CONCLUSIONS: AA amyloidosis is associated with higher risk of mortality after kidney transplantation. Inflammatory indicators should be monitored closely, and persistent high levels of acute-phase reactants should raise concerns about amyloid recurrence in allograft.


Assuntos
Amiloidose/cirurgia , Febre Familiar do Mediterrâneo/complicações , Rejeição de Enxerto/mortalidade , Falência Renal Crônica/cirurgia , Transplante de Rim/efeitos adversos , Adulto , Aloenxertos/imunologia , Aloenxertos/patologia , Amiloidose/imunologia , Amiloidose/mortalidade , Amiloidose/patologia , Biópsia , Febre Familiar do Mediterrâneo/imunologia , Febre Familiar do Mediterrâneo/mortalidade , Febre Familiar do Mediterrâneo/cirurgia , Feminino , Seguimentos , Rejeição de Enxerto/imunologia , Rejeição de Enxerto/patologia , Sobrevivência de Enxerto/imunologia , Humanos , Estimativa de Kaplan-Meier , Rim/imunologia , Rim/patologia , Falência Renal Crônica/imunologia , Falência Renal Crônica/mortalidade , Falência Renal Crônica/patologia , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Proteína Amiloide A Sérica/imunologia , Proteína Amiloide A Sérica/metabolismo , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
2.
World Neurosurg ; 140: 325-331, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32461181

RESUMO

BACKGROUND: Solitary spinal amyloidoma (SSA) is a rare and poorly characterized disease. There are few cases described, and the knowledge of this neoplasm is limited. A more accurate description of demographics, clinical findings, and outcomes may be useful for a better understanding of this pathology, as well as therapeutic intervention, adding value to the research of localized amyloidosis. METHODS: A systematic search was carried out from when registries began until February 2020. We also include a case diagnosed and treated in our department. Descriptive statistics were used to evaluate data, demographics, clinical findings, diagnostic modalities, therapeutics, and finally neurologic outcomes. The Kaplan-Meier method was used to assess overall survival and progression-free survival. RESULTS: The final cohort comprises 35 patients. The mean age at diagnosis was 61.97 years, and 68.60% of the patients were male. SSA developed more frequently in the thoracic spine (48.60%), followed by the cervical spine (17.10%). Intradural lesions were rare, and the average neoplastic score for spinal instability was 9.5 points. The most common symptoms were impaired motor function (74.29%) and axial back pain (65.70%). After surgery, neurologic recovery was reported in 82.90% of cases. Mean progression-free survival and mean overall survival were 47.26 and 156.66 months. CONCLUSIONS: SSA is a rare subgroup of localized amyloidosis, usually being diagnosed in male patients between the sixth and eighth decades. The gold standard treatment seems to be surgical resection. SSA patients have excellent long-term survival and a low rate of local recurrence.


Assuntos
Amiloidose/cirurgia , Coluna Vertebral/cirurgia , Amiloidose/patologia , Feminino , Humanos , Masculino , Doenças da Coluna Vertebral/patologia , Doenças da Coluna Vertebral/cirurgia , Coluna Vertebral/patologia , Resultado do Tratamento
4.
J Oral Maxillofac Surg ; 78(9): 1572-1582, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32442425

RESUMO

PURPOSE: Localized amyloidosis of the tongue is a benign condition in which surgical management may be considered. The aim of the study was to review the current literature and report a case. MATERIALS AND METHODS: We searched the PubMed database for all relevant articles reporting cases of localized tongue amyloidosis published between 1980 and February 2020. In addition, we updated 1 case diagnosed and treated in our department. RESULTS: A 49-year-old male patient presented with an asymptomatic tongue nodule of the dorsum mimicking median rhomboid glossitis. The results of an incisional biopsy showed an amyloid on Congo red staining and positive findings for the κ light chain by immunohistochemical analysis. The findings of the systemic workup were negative. Therefore, a diagnosis of localized κ light-chain amyloidosis was made. The patient underwent a resection of the lesion, and no recurrence or progression was observed during a period of 18 months. The literature review showed 12 reports describing 21 patients (11 men, 52.3%) with localized tongue amyloidosis. The most common clinical presentation was nodular with a single lesion of the tongue dorsum (15 patients, 71.4%). All cases showed positive findings on Congo red staining. Immunohistochemical analysis findings were available for only 9 patients (42.8%) and showed light-chain amyloidosis. No case showed any systemic involvement or the development of systemic disease. Surgical excision was performed in 9 cases, with recurrence at the site of operation in 2 cases. CONCLUSIONS: Localized amyloidosis of the tongue is a rare disease in which surgical excision may be therapeutic when a multidisciplinary evaluation does not show any systemic disease. We recommend an excision when the lesion is persistent or shows an enlargement or when discomfort is reported. In the case of any further local recurrence, resection may be repeated.


Assuntos
Amiloidose , Doenças da Língua , Amiloide , Amiloidose/diagnóstico , Amiloidose/cirurgia , Biópsia , Humanos , Masculino , Pessoa de Meia-Idade , Língua/cirurgia , Doenças da Língua/diagnóstico , Doenças da Língua/cirurgia
5.
Curr Opin Organ Transplant ; 25(3): 211-217, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32304420

RESUMO

PURPOSE OF REVIEW: To deeply investigate one type of intracellular cardiac storage disease, the Fabry disease and one example of extracellular cardiac storage disease, the cardiac amyloidosis, with the aim to collect data about cardiac treatment at the end-stage level and the feasibility of heart transplantation (HTx) in this setting of patients. RECENT FINDINGS: Some registry describes that Fabry disease and cardiac amyloidosis showed similar characteristics as hypertrophic cardiomyopathies; thus, their correct diagnosis is often missing or time consuming. A multiorgan approach is mandatory to recognize the main systemic findings of these diseases, involving also the kidneys, the brain, the autonomous system and the skin. SUMMARY: The early diagnosis of these diseases is required to start as soon as possible the correct therapy for the patients affected. However, the end-stage heart failure is common and HTx could be offered to selected patients, especially if affected by light chain cardiac amyloidosis, to allow to perform the autologous stem cell transplantation after the cardiac transplant. Considering the Fabry disease patients, the enzyme recombinant therapy is also mandatory after HTx to avoid potential release of the systemic disease on the donor graft.


Assuntos
Amiloidose/cirurgia , Doença de Fabry/cirurgia , Cardiopatias/cirurgia , Transplante de Coração/métodos , Adulto , Idoso , Transplante de Células-Tronco Hematopoéticas , Humanos , Pessoa de Meia-Idade , Transplante Autólogo
6.
J Clin Ultrasound ; 48(3): 168-173, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32003472

RESUMO

We describe the case of a 41-year-old woman with primary Sjögren's syndrome (SS) who presented multiple recurrences of breast amyloidosis. Each recurrence of breast amyloidosis showed different sonographic features, potentially mimicking malignancy. We briefly discuss the possible cause of this variability in imaging features based on the radiologic-histologic correlation.


Assuntos
Amiloidose/complicações , Amiloidose/diagnóstico por imagem , Doenças Mamárias/complicações , Doenças Mamárias/diagnóstico por imagem , Síndrome de Sjogren/complicações , Adulto , Amiloidose/patologia , Amiloidose/cirurgia , Doenças Mamárias/patologia , Doenças Mamárias/cirurgia , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Recidiva , Ultrassonografia/métodos
7.
World Neurosurg ; 138: 205-213, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32084617

RESUMO

BACKGROUND: Management of cerebral amyloidomas has lacked consensus owing to their rarity. We present a case and review 39 reported cases of amyloidomas in the literature, comparing their initial presentation, imaging characteristics, treatment, and progression. CASE DESCRIPTION: A 56-year-old woman presented with a history of intractable headaches and progressive speech difficulty. Imaging demonstrated a 1.7 × 2.6 cm left frontal subcortical hemorrhagic mass with an associated developmental venous anomaly, characteristics initially suggestive of a cavernous malformation. Diffusion tractography imaging was employed to avoid interference with the intact arcuate fasciculus and corticospinal tracts to minimize neurologic deficits. CONCLUSIONS: To our knowledge, this is the first documented case of the use of diffusion tractography in preoperative planning for cerebral amyloidoma resection. Our systematic review of 39 reported cases of amyloidomas in the literature provided insight regarding how these rare lesions have manifested and progressed and further understanding of current theories regarding their etiology and pathophysiology. Cerebral amyloidomas are rare, localized, proteinaceous aggregates with variable presentation and prognosis and no apparent relationship to systemic amyloidosis. Biopsy may be useful in determination of progression. Diffusion tractography is a valuable tool for minimizing complications associated with resection.


Assuntos
Amiloidose/cirurgia , Encefalopatias/cirurgia , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Procedimentos Neurocirúrgicos/métodos , Feminino , Lobo Frontal/patologia , Lobo Frontal/cirurgia , Humanos , Pessoa de Meia-Idade
8.
Cardiovasc Pathol ; 46: 107191, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31927216

RESUMO

Pericardial amyloidosis is a rare cause of pericardial effusion. Here, we report a case of recurrent pericardial effusion because of pericardial amyloid deposition. The patient was a man in his 40s admitted for pulmonary embolism. During hospitalization, arterial fibrillation and cardiac tamponade were observed, and an initial pericardial puncture was performed. Thereafter, pericardial puncture was repeated nine times over the next two years. Cytological examination of the pericardial effusion suggested malignant mesothelioma. Afterward, pericardial fenestration and partial resection were performed. Intraoperatively, a thickened pericardium and hemorrhagic pericardial effusion were noted. Histologically, the surface of the pericardium was covered by an eosinophilic amorphous material. Congo red and DYLON stains, electron microscopy, and immunohistochemical findings revealed localized amyloidosis composed of an immunoglobulin lambda light chain. Although the patient did not receive further treatment for 5 years postoperatively, his renal and cardiac functions remained within normal limits. Based on these findings, the patient was diagnosed with localized amyloidosis. So far, hemorrhagic pericardial effusion has been reported in few cases with systemic amyloidosis. Because localized immunoglobulin light-chain-derived (AL) amyloidosis may progress to systemic disease (although it is a very rare occurrence), long-term follow-up is necessary to detect recurrence or progression to a systemic form.


Assuntos
Amiloidose/complicações , Cardiopatias/complicações , Derrame Pericárdico/etiologia , Pericárdio/patologia , Amiloidose/metabolismo , Amiloidose/patologia , Amiloidose/cirurgia , Progressão da Doença , Cardiopatias/metabolismo , Cardiopatias/patologia , Cardiopatias/cirurgia , Humanos , Masculino , Derrame Pericárdico/metabolismo , Derrame Pericárdico/patologia , Derrame Pericárdico/cirurgia , Pericardiectomia , Pericárdio/metabolismo , Pericárdio/cirurgia , Recidiva , Resultado do Tratamento
10.
Klin Monbl Augenheilkd ; 237(1): 35-40, 2020 Jan.
Artigo em Alemão | MEDLINE | ID: mdl-31968364

RESUMO

BACKGROUND: Primary localised orbital amyloidosis (PLOA) is a very rare disease. In contrast to the isolated manifestation, systemic involvement can be associated with potentially life-threatening consequences. However, the isolated involvement of the orbit can also lead to serious complications. MATERIAL AND METHODS: Two cases of PLOA are described and the necessary ophthalmic, internistic and immunohistochemical diagnostic testing are explained. RESULTS: The first case describes a 71-year-old woman with PLOA. In the clinic, a yellow-orange bumpy prominence in the nasal lower quadrant without further ophthalmological abnormalities was found. Extensive diagnostic testing found no systemic manifestation. The patient herself was free of complaints. In the follow-up over 4 years, patient showed slow progression without ocular complications. The second case is a 72-year-old male patient with similar clinical signs but localisation in the temporal superior quadrant. During the clinical course, multiple ophthalmological complications developed (ptosis, protrusio bulbi, diplopia, secondary glaucoma, perforated corneal ulcer in neurotrophic keratopathy). Perforating keratoplasty had to be performed. Fractioned radiotherapy led to stabilisation of the disease. The follow-up period was 4 years. CONCLUSION: PLOA can lead to visual and organ threatening complications. Accurate diagnosis is required for further diagnostic and therapeutic procedures and to counteract potential local and systemic complications. Interindividual differences in the course have to be considered.


Assuntos
Amiloidose , Blefaroptose , Exoftalmia , Ceratite , Idoso , Amiloidose/diagnóstico , Amiloidose/cirurgia , Blefaroptose/diagnóstico , Blefaroptose/cirurgia , Exoftalmia/diagnóstico , Exoftalmia/cirurgia , Feminino , Humanos , Ceratite/diagnóstico , Ceratite/cirurgia , Masculino , Órbita
11.
Orbit ; 39(1): 13-17, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31577164

RESUMO

Purpose: To investigate the natural history of ocular adnexal and orbital amyloidosis.Methods: In a retrospective, non-comparative case series, the clinical records of patients with biopsy-proven ocular, adnexal, and orbital amyloidosis managed at our institution between 1980 and 2016 were evaluated.Results: Forty-one patients (29 female; 71%) were identified. The mean interval from presentation to diagnosis was 24 months (median 12 months, range 1-84 months). Whilst most patients presented with a conjunctival mass (34/41; 83%) or ptosis (15/41; 37%), the diagnosis was not immediately evident in all - two patients had 3 ptosis operations prior to obtaining a tissue biopsy that revealed amyloid deposition. Three-quarters (31/41; 76%) of patients had localised primary ocular adnexal and orbital amyloidosis, 4 (10%) had associated systemic disease, and 6 (15%) were found to have underlying haematological malignancy on further investigation. During a mean follow-up of 8 years (median 7 years; range 6 months - 36 years), 2 (5%) patients lost vision, 21 (51%) had surgical intervention other than biopsy, and 2 (5%) had local radiotherapy for amyloid deposition secondary to lymphoproliferative disease.Conclusions: The varied presentations of ocular adnexal and orbital amyloidosis and the need for confirmatory biopsy often leads to a significant delay between first symptoms and diagnosis. While rarely sight-threatening, ocular adnexal and orbital amyloidosis carries significant morbidities and has a systemic association in a quarter of patients.


Assuntos
Amiloidose/diagnóstico , Doenças da Túnica Conjuntiva/diagnóstico , Doenças Palpebrais/diagnóstico , Doenças Orbitárias/diagnóstico , Adulto , Distribuição por Idade , Amiloidose/epidemiologia , Amiloidose/cirurgia , Biópsia por Agulha , Estudos de Coortes , Doenças da Túnica Conjuntiva/epidemiologia , Doenças da Túnica Conjuntiva/cirurgia , Doenças Palpebrais/cirurgia , Feminino , Finlândia , Humanos , Imuno-Histoquímica , Incidência , Masculino , Pessoa de Meia-Idade , Doenças Orbitárias/epidemiologia , Doenças Orbitárias/cirurgia , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Distribuição por Sexo , Tomografia Computadorizada por Raios X/métodos , Adulto Jovem
13.
Vestn Otorinolaringol ; 84(4): 48-50, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31579058

RESUMO

Amyloidosis of the larynx is rare in the pediatric age group. We present a clinical case of laryngeal amyloidosis manifesting as false vocal fold bulging in a thirteen-year-old girl with hoarseness and with initial suspicion of laryngeal tumor. Computed tomography and ultrasound scanning of the larynx were useful in determining the laryngeal process size along with fibro laryngoscopy. There was performed a microsurgical removal of the larynx formation. The results of Congo red staining of the formation were characteristic of amyloid. Systemic manifestations of the disease were not revealed.


Assuntos
Amiloidose , Doenças da Laringe , Adolescente , Amiloidose/complicações , Amiloidose/diagnóstico , Amiloidose/cirurgia , Criança , Diagnóstico Diferencial , Feminino , Rouquidão , Humanos , Doenças da Laringe/complicações , Doenças da Laringe/diagnóstico , Doenças da Laringe/cirurgia , Neoplasias Laríngeas/diagnóstico , Laringoscopia
15.
Medicine (Baltimore) ; 98(35): e16830, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31464908

RESUMO

RATIONALE: Amyloidosis accounts for 2% of head and neck tumors. Amyloidosis that develops in the head and neck region is localized amyloidosis. Multifocal amyloidosis in the head and neck region is extremely rare. PATIENT CONCERNS: The patient presented to the clinic of otolaryngology with nasal obstruction, anosmia and left neck mass for several months. DIAGNOSIS: A left nasopharynx tumor was revealed under nasopharyngeal scope. Eosinophilic, proteinaceous material was revealed under a pathology scope in the nasopharynx tissue and neck tumor. Congo red staining demonstrated pale congophilic amorphous material with apple-green birefringence under cross-polarized light, and multifocal amyloidosis was diagnosed. Amyloidosis secondary to systemic lupus erythematosus (SLE) was confirmed after a series of investigations. INTERVENTIONS: The patient underwent local excision for multifocal amyloidosis without following management. To control underlying SLE, the patient accepted steroid pulse therapy and immunosuppressants. The patient eventually achieved disease remission. OUTCOMES: During the 6 months of follow-up in the outpatient department of otolaryngology and rheumatology, complications, recurrence of nasopharyngeal amyloidosis, and SLE flare-up were not observed. LESSONS: Head and neck amyloidosis involving the nasopharynx is a rare presentation of this disease. Head and neck multifocal amyloidosis should be taken as a hint of systemic disease. In head and neck amyloidosis, a comprehensive survey should be performed to clarify the underlying disease predisposing to amyloidosis and organ involvement.


Assuntos
Amiloidose/etiologia , Neoplasias de Cabeça e Pescoço/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Neoplasias Nasofaríngeas/diagnóstico , Adulto , Amiloidose/cirurgia , Feminino , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Neoplasias Nasofaríngeas/patologia , Neoplasias Nasofaríngeas/cirurgia , Indução de Remissão , Esteroides/uso terapêutico , Resultado do Tratamento
16.
Transplant Proc ; 51(7): 2292-2294, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31400972

RESUMO

Familial Mediterranean fever (FMF) is an important and preventable cause of chronic kidney disease due to secondary amyloidosis. Although colchicine is the first-line therapy in patients with FMF with 60% to 65% complete remission rates, 5% to 10% of patients are colchicine-resistant and 5% to 10% of them are intolerant to the therapy. Anti-interleukin-1 agents, such as anakinra and canakinumab, are safe and efficient therapeutic options in patients with colchicine resistance or intolerance. However, the data on management of these targeted agents is limited in recipients of kidney transplant (RKT). In this case series, we aim to share our experience on canakinumab therapy of 4 RKTs with FMF-related amyloidosis, who were followed up in our clinic between 2010 and 2017. All of the 4 patients with end-stage renal disease were colchicine- resistant and on other alternative therapies, which provided poor disease control. For efficient control of secondary amyloidosis, canakinumab therapy was initiated in 1 of the patients before the renal transplant, and for the remaining patients after renal transplant. Any serious adverse effect, development of proteinuria, or graft dysfunction has not been observed in any of the patients. Under the canakinumab treatment, complete clinical responses, prevent typical familial Mediterranean fever attacks with fever and arthritis and abdominal pain, normalized serum amyloid A and C-reactive protein levels were achieved in all patients. Canakinumab treatment is a safe and effective therapeutic option for RKTs with FMF who are resistant or intolerant to colchicine and anakinra.


Assuntos
Amiloidose/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Febre Familiar do Mediterrâneo/tratamento farmacológico , Falência Renal Crônica/tratamento farmacológico , Transplante de Rim , Adulto , Amiloidose/complicações , Amiloidose/cirurgia , Febre Familiar do Mediterrâneo/complicações , Febre Familiar do Mediterrâneo/cirurgia , Feminino , Humanos , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Masculino , Período Pós-Operatório , Resultado do Tratamento
18.
Am J Case Rep ; 20: 933-936, 2019 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-31256189

RESUMO

BACKGROUND Intracardiac thrombosis has been known to be associated with not only hepatocellular carcinoma but also with amyloidosis and use of a cardiac implantable electronic device. We report a case of a continuous tumor thrombus with hepatocellular carcinoma from the portal vein and hepatic vein to the right atrium via the inferior vena cava in a patient with a cardiac amyloidosis and an implanted cardiac resynchronization therapy (CRT) device. CASE REPORT A 68-year-old female first admitted to our hospital because of heart failure with an AL type primary cardiac amyloidosis. After 3 years, she underwent an implantation of a CRT device for biventricular pacing following repeated episodes of heart failure and low left ventricular ejection fraction of 34% with NYHA class III. Again, she presented with symptoms of heart failure and cardiomegaly on chest x-ray at 7 years after the CRT device implantation. The echocardiography showed a huge echogenic mass occupying the right atrium, and 64 multi-detector computed tomography showed a lobulated heterogeneously enhancing mass of hepatocellular carcinoma in the right upper lobe of her liver and a continuous tumor thrombus from the portal vein and hepatic vein to the right atrium via the inferior vena cava. CONCLUSIONS Intracardiac thrombosis and heart failure occurred in a patient with hepatocellular carcinoma and cardiac amyloidosis, who had an implanted CRT device, which resulted not only in hypercoagulability by the hepatocellular carcinoma itself and the accumulation of various risk factors, but also the progression of myocardial damage with the development of amyloidosis.


Assuntos
Amiloidose/complicações , Carcinoma Hepatocelular/complicações , Cardiopatias/complicações , Insuficiência Cardíaca/complicações , Neoplasias Hepáticas/complicações , Trombose/complicações , Idoso , Amiloidose/cirurgia , Dispositivos de Terapia de Ressincronização Cardíaca , Ecocardiografia , Feminino , Cardiopatias/cirurgia , Insuficiência Cardíaca/cirurgia , Humanos , Fatores de Risco , Trombofilia , Trombose/cirurgia , Tomografia Computadorizada por Raios X
19.
Neuropathology ; 39(3): 231-239, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31044465

RESUMO

Of the myriad of variants of amyloidoses where abnormally folded proteins damage native tissue, primary cervical spine amyloidoma represents one of the rarest forms. Since clinical presentations and imaging findings appear similar to other pathologies, including abscesses, metastatic lesions, and inflammatory lesions, a definitive diagnosis requires a biopsy with specific immunohistochemical stains. We present the first known case of primary cervical amyloid light-chain (AL)-κ subtype amyloidoma and compare the clinical presentations, imaging findings, treatment options, and immunohistochemical subtypes of primary, hemodialysis, and multiple myeloma cervical amyloidomas. Our case is of a 58-year-old man who developed neck pain radiating to the left arm with bilateral upper extremity weakness over several months. Magnetic resonance imaging revealed a circumferential C1-C2 mass extending into the neural foramina inducing severe mass effect. The patient underwent C2 laminectomy and resection of the lesion which was discovered during surgery to be completely epidural. Postoperatively, his pain and weakness improved. A complete work-up was negative for systemic amyloidosis or inflammatory conditions. In the setting of a long clinical history of hemodialysis, this patient required specific staining and laboratory testing to correctly diagnose his primary cervical AL-κ subtype amyloidoma. Cervical amyloidomas comprise a very small minority of amyloid pathology with an exceptional prognosis following successful surgical resection and stabilization. It is recommended these patients undergo surgical resection with appropriate characterization and a complete work-up to rule out systemic disease.


Assuntos
Amiloide , Amiloidose/diagnóstico por imagem , Amiloidose/cirurgia , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Amiloide/isolamento & purificação , Humanos , Masculino , Pessoa de Meia-Idade
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