• Title/Summary/Keyword: amyloidosis

Search Result 67, Processing Time 0.025 seconds

Light-Chain Cardiac Amyloidosis: Cardiac Magnetic Resonance for Assessing Response to Chemotherapy

  • Yubo Guo;Xiao Li;Yajuan Gao;Kaini Shen;Lu Lin;Jian Wang;Jian Cao;Zhuoli Zhang;Ke Wan;Xi Yang Zhou;Yucheng Chen;Long Jiang Zhang;Jian Li;Yining Wang
    • Korean Journal of Radiology
    • /
    • v.25 no.5
    • /
    • pp.426-437
    • /
    • 2024
  • Objective: Cardiac magnetic resonance (CMR) is a diagnostic tool that provides precise and reproducible information about cardiac structure, function, and tissue characterization, aiding in the monitoring of chemotherapy response in patients with light-chain cardiac amyloidosis (AL-CA). This study aimed to evaluate the feasibility of CMR in monitoring responses to chemotherapy in patients with AL-CA. Materials and Methods: In this prospective study, we enrolled 111 patients with AL-CA (50.5% male; median age, 54 [interquartile range, 49-63] years). Patients underwent longitudinal monitoring using biomarkers and CMR imaging. At follow-up after chemotherapy, patients were categorized into superior and inferior response groups based on their hematological and cardiac laboratory responses to chemotherapy. Changes in CMR findings across therapies and differences between response groups were analyzed. Results: Following chemotherapy (before vs. after), there were significant increases in myocardial T2 (43.6 ± 3.5 ms vs. 44.6 ± 4.1 ms; P = 0.008), recovery in right ventricular (RV) longitudinal strain (median of -9.6% vs. -11.7%; P = 0.031), and decrease in RV extracellular volume fraction (ECV) (median of 53.9% vs. 51.6%; P = 0.048). These changes were more pronounced in the superior-response group. Patients with superior cardiac laboratory response showed significantly greater reductions in RV ECV (-2.9% [interquartile range, -8.7%-1.1%] vs. 1.7% [-5.5%-7.1%]; P = 0.017) and left ventricular ECV (-2.0% [-6.0%-1.3%] vs. 2.0% [-3.0%-5.0%]; P = 0.01) compared with those with inferior response. Conclusion: Cardiac amyloid deposition can regress following chemotherapy in patients with AL-CA, particularly showing more prominent regression, possibly earlier, in the RV. CMR emerges as an effective tool for monitoring associated tissue characteristics and ventricular functional recovery in patients with AL-CA undergoing chemotherapy, thereby supporting its utility in treatment response assessment.

Regional Amyloid Burden Differences Evaluated Using Quantitative Cardiac MRI in Patients with Cardiac Amyloidosis

  • Jin Young Kim;Yoo Jin Hong;Kyunghwa Han;Hye-Jeong Lee;Jin Hur;Young Jin Kim;Byoung Wook Choi
    • Korean Journal of Radiology
    • /
    • v.22 no.6
    • /
    • pp.880-889
    • /
    • 2021
  • Objective: This study aimed to investigate the regional amyloid burden and myocardial deformation using T1 mapping and strain values in patients with cardiac amyloidosis (CA) according to late gadolinium enhancement (LGE) patterns. Materials and Methods: Forty patients with CA were divided into 2 groups per LGE pattern, and 15 healthy subjects were enrolled. Global and regional native T1 and T2 mapping, extracellular volume (ECV), and cardiac magnetic resonance (CMR)-feature tracking strain values were compared in an intergroup and interregional manner. Results: Of the patients with CA, 32 had diffuse global LGE (group 2), and 8 had focal patchy or no LGE (group 1). Global native T1, T2, and ECV were significantly higher in groups 1 and 2 than in the control group (native T1: 1384.4 ms vs. 1466.8 ms vs. 1230.5 ms; T2: 53.8 ms vs. 54.2 ms vs. 48.9 ms; and ECV: 36.9% vs. 51.4% vs. 26.0%, respectively; all, p < 0.001). Basal ECV (53.7%) was significantly higher than the mid and apical ECVs (50.1% and 50.0%, respectively; p < 0.001) in group 2. Basal and mid peak radial strains (PRSs) and peak circumferential strains (PCSs) were significantly lower than the apical PRS and PCS, respectively (PRS, 15.6% vs. 16.7% vs. 26.9%; and PCS, -9.7% vs. -10.9% vs. -15.0%; all, p < 0.001). Basal ECV and basal strain (2-dimensional PRS) in group 2 showed a significant negative correlation (r = -0.623, p < 0.001). Group 1 showed no regional ECV differences (basal, 37.0%; mid, 35.9%; and apical, 38.3%; p = 0.184). Conclusion: Quantitative T1 mapping parameters such as native T1 and ECV may help diagnose early CA. ECV, in particular, can reflect regional differences in the amyloid deposition in patients with advanced CA, and increased basal ECV is related to decreased basal strain. Therefore, quantitative CMR parameters may help diagnose CA and determine its severity in patients with or without LGE.

Fine Needle Aspiration Cytology of Amyloid Goiter - A Case Report - (아밀로이드 갑상선종대의 세침흡인 세포학적 소견 - 1예 보고 -)

  • Chae, Seoung-Wan;Nam, Eun-Sook;Kim, Duck-Hwan;Shin, Hyung-Sik;Sohn, Jin-Hee
    • The Korean Journal of Cytopathology
    • /
    • v.11 no.2
    • /
    • pp.109-114
    • /
    • 2000
  • Amyloid golfer is a rare disease entity that is defined as a symptomatic mass or clinically detectable thyroid enlargement because of amyloid deposition. We present a case of amyloid golfer diagnosed in the fine needle aspiration cytology(FNAC) in a 73-year-old Korean woman presented with nephrotic syndrome and thyroid enlargement. The thyroid function was in normal range. Thyroid scan showed a nodule, $4{\time}2cm$ in the right lobe with underlying diffuse golfer. Aspirates revealed benign looking follicular cells and scattered eosinophilic material. The sections of the cell block showed nodular deposit of eosinophilic hyalinized material in the interfollicular area. It showed apple-green birefringence under polarization with Congo red stain. The renal biopsy also exhibited deposition of eosinophilic materials in the glomeruli and interstitial vascular wall, which were confirmed as amyloidosis. This material was morphologically distinct from the colloid.

  • PDF

Calcaneal Insufficiency Avulsion Fracture in a Well-Controlled Type 2 Diabetic Patient: A Case Report

  • Kim, Seong-Tae;Moon, Myung-Sang;Kwon, Ki-Tae;Park, Bong-Keun;Ha, Chang Won;Ahn, Jungtae
    • Journal of Korean Foot and Ankle Society
    • /
    • v.19 no.2
    • /
    • pp.73-76
    • /
    • 2015
  • The calcaneal insufficiency avulsion fracture usually occurs in an area of fused apophysis in adults without significant history of trauma or overuse activities. It is an uncommon injury which has been described in patients with complicated diabetes, Charcot neuroarthropathy, amyloidosis with neuropathy, severe osteoporosis, and other conditions. Discussion of the issue of fracture location is still not sufficient. We report on a case of a 50-year-old male who experienced a non-traumatic diabetic calcaneal insufficiency fracture. Intraoperatively, a biopsy specimen was obtained from the exposed fracture site for histological study. We assume that the calcaneal fused apophyseal line is the weak point of failure due to various incomplete mixtures of trabecular bone, woven bone, and cartilaginous tissues, and may fail when repeated tensile stress is imposed.

Heart Transplantation in a 78-year-old Patient (78세 고령에서의 심장이식)

  • Park, Byung-Joon;Park, Pyo-Won;Choi, Seon-Uoo;Sung, Ki-Ick;Yang, Ji-Hyuk
    • Journal of Chest Surgery
    • /
    • v.41 no.5
    • /
    • pp.640-642
    • /
    • 2008
  • Advanced age is known to be a risk factor for early mortality after heart transplantation and is considered to be a relative contraindication. However, recent studies have shown that there are no significant differences in early and midterm survival rates between older and younger recipients. With rising life expectancy and improvements in medical support, the demand for heart transplantation in elderly patients continues to grow. We present a successful case of heart transplantation in a 78-year-old patient.

A Case of Plasma Cell Mucositis Arising From the Larynx (후두에 발생한 형질 세포 점막염 1예)

  • Lee, Chang Bae;Heo, Jae Won;Pak, Min Gyoung;Lee, Dong Kun
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
    • /
    • v.32 no.2
    • /
    • pp.98-103
    • /
    • 2021
  • Plasma cell mucositis is a very rare benign disease characterized by dense lymphoplasmacytic infiltration in the submucosa layer. It appears as a reddish ulcer on the mucous membrane or as a cobblestone or nodular mass on the affected mucosa. When it involves the pharynx or larynx, the patient presents with dysphagia, voice change and dyspnea. Clinically, it is important to differentiate with malignant diseases such as extramedullary plasmacytoma, amyloidosis and sarcodosis. Several cases of mucositis in the larynx have been reported in English literature, but none have been reported in Korea. We report a case of plasma cell mucositis in the larynx with a review of literature.

Differential Diagnosis of Thick Myocardium according to Histologic Features Revealed by Multiparametric Cardiac Magnetic Resonance Imaging

  • Min Jae Cha;Cherry Kim;Chan Ho Park;Yoo Jin Hong;Jae Min Shin;Tae Hoon Kim;Yoon Jin Cha;Chul Hwan Park
    • Korean Journal of Radiology
    • /
    • v.23 no.6
    • /
    • pp.581-597
    • /
    • 2022
  • Left ventricular (LV) wall thickening, or LV hypertrophy (LVH), is common and occurs in diverse conditions including hypertrophic cardiomyopathy (HCM), hypertensive heart disease, aortic valve stenosis, lysosomal storage disorders, cardiac amyloidosis, mitochondrial cardiomyopathy, sarcoidosis and athlete's heart. Cardiac magnetic resonance (CMR) imaging provides various tissue contrasts and characteristics that reflect histological changes in the myocardium, such as cellular hypertrophy, cardiomyocyte disarray, interstitial fibrosis, extracellular accumulation of insoluble proteins, intracellular accumulation of fat, and intracellular vacuolar changes. Therefore, CMR imaging may be beneficial in establishing a differential diagnosis of LVH. Although various diseases share LV wall thickening as a common feature, the histologic changes that underscore each disease are distinct. This review focuses on CMR multiparametric myocardial analysis, which may provide clues for the differentiation of thickened myocardium based on the histologic features of HCM and its phenocopies.

Migrating Lobar Atelectasis of the Right Lung: Radiologic Findings in Six Patients

  • Tae Sung Kim;Kyung Soo Lee;Jung Hwa Hwang;In Wook Choo;Jae Hoon Lim
    • Korean Journal of Radiology
    • /
    • v.1 no.1
    • /
    • pp.33-37
    • /
    • 2000
  • Objective: To describe the radiologic findings of migrating lobar atelectasis of the right lung. Materials and Methods: Chest radiographs (n = 6) and CT scans (n = 5) of six patients with migrating lobar atelectasis of the right lung were analyzed retrospectively. The underlying diseases associated with lobar atelectasis were bronchogenic carcinoma (n = 4), bronchial tuberculosis (n = 1), and tracheobronchial amyloidosis (n = 1). Results: Atelectasis involved the right upper lobe (RUL) (n = 3) and both the RUL and right middle lobe (RML) (n = 3). On supine anteroposterior radiographs (n = 5) and on an erect posteroanterior radiograph (n = 1), the atelectatic lobe(s) occupied the right upper lung zone, with a wedge shape abutting onto the right mediastinal border. On erect posteroanterior radiographs (n = 6), the heavy atelectatic lobe(s) migrated downward, forming a peri- or infrahilar area of increased opacity and obscuring the right cardiac margin. Erect lateral radiographs (n = 4) showed inferior shift of the anterosuperiorly located atelectatic lobe(s) to the anteroinferior portion of the hemithorax. Conclusion: Atelectatic lobe(s) can move within the hemithorax according to changes in a patient s position. This process involves the RUL or both the RUL and RML.

  • PDF

A Case of ANCA-associated Pauci-immune Crescentic Glomerulonephritis in Juvenile Rheumatoid Arthritis (소아기 류마티스 관절염에서 발견된 ANCA 연관 극소면역성 반월상 사구체신염 1례)

  • Hwang You Sik;Rhie Young Jun;Ahn Sun Young;Kim Dong Soo;Lee Jae Seung;Jeong Hyun Joo
    • Childhood Kidney Diseases
    • /
    • v.9 no.2
    • /
    • pp.231-236
    • /
    • 2005
  • Juvenile rheumatoid arthritis(JRA) is the most common major connective tissue disease in children. Renal involvement in JRA is rare. Among the renal lesions that have been reported in JRA, amyloidosis and drug-induced nephropathy are the most common. Crescentic glomerulonephritis in JRA has rarely been reported. We report a case of ANCA-associated pauci-immune crescentic glomerulonephritis in JRA. The patient was a 15-year old boy with a 3-year history of JRA. He presented with gross hematuria, proteinuria, positive p-ANCA and elevation of BUN and creatinine. Pathologic findings revealed focal necrotizing and crescentic glomerulonephritis. There were no significant immunoglobulin or complement deposits. His renal function recovered after intravenous methylprednisolone pulse therapy and oral steroid use. In Korea, this is the first reported case of pauci-immune crescentic glomerulonephritis in JRA. (J Korean Soc Pediatr Nephrol 2005;9:231-236)

  • PDF