Cardiac nonamyloidotic immunoglobulin deposition disease

Toor, Amir A.; Ramdane, Ben A; Joseph, Jacob; Thomas, Maria; O’Hara, Carl; Barlogie, Bart; Walker, Patrick D.; Joseph, Lija

doi:10.1038/modpathol.3800524

Cited by 38 publications

(19 citation statements)

References 14 publications

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“…The most common clinical presentation is the result of kidney involvement which is due to progressive accumulation of light chains from plasma filtration and includes proteinuria, nephrotic syndrome and/or renal failure [1][2][3][4]. Symptomatic extrarenal deposition is rare and has been described in the heart [10,11], liver [2], lungs, joints [12], central and peripheral nervous system [5]. The lung involvement may rarely dominate the clinical course of LCDD and lead to severe respiratory insufficiency requiring lung transplantation [13][14][15].…”

Section: Discussionmentioning

confidence: 99%

Extraosseus Plasmacytoma of the Pharynx with Localized Light Chain Deposition. Case Report

Zidar

Zver

Jurčić

2009

Pathol. Oncol. Res.

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Light chain deposition disease (LCDD) is a rare disorder associated with a clonal proliferation of plasma cells, which synthesize abnormal monoclonal immunoglobulin light chains. It is characterized by systemic deposition of light chains in various organs, with the kidneys being most commonly affected. There have been few reports of isolated LCDD, i.e. in the brain, lungs and cervical lymph nodes. We here report on another patient with an isolated form of LCDD, which was limited to the pharyngeal mucosa and was associated with an extraosseus plasmacytoma of the pharynx, expanding the spectrum that has been recognized for LCDD. The patient was treated by local radiotherapy, with an excellent response. A less aggressive clinical course can probably be expected than in the usual form of LCDD, but a long-term follow-up is necessary to establish the clinical significance of this variant of LCDD.

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Section: Discussionmentioning

confidence: 99%

Extraosseus Plasmacytoma of the Pharynx with Localized Light Chain Deposition. Case Report

Zidar

Zver

Jurčić

2009

Pathol. Oncol. Res.

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“…In patients with positive findings, the type of amyloid was determined by immunofluorescence of cryostat sections of frozen tissue by staining with polyclonal antibodies against light chains (kappa and lambda), and TTR [14].…”

Section: Histologymentioning

confidence: 99%

Pilot study for left ventricular imaging phenotype of patients over 65 years old with heart failure and preserved ejection fraction: the high prevalence of amyloid cardiomyopathy

Smires

Victor

Ribes

et al. 2016

Int J Cardiovasc Imaging

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This study sought to phenotype patients over 65 years old with heart failure and preserved ejection fraction (HFpEF) using clinical available comprehensive cardiovascular imaging modalities. Forty-nine patients with HFpEF and without coronary artery disease underwent clinical evaluation, electrocardiography, echocardiography, cardiac magnetic resonance (CMR) and Tc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy (Tc-DPD). The mean population age was 76 ± 8 years. Most of the patients (53 %) were NYHA class II. Mean NT-Pro-NBNP level was 1961 ± 2372 pg/ml. CMR exhibited a hypertrophic cardiomyopathy or infiltrative pattern in 3 (6 %) and 15 (31 %) patients, respectively. In the latter subgroup, Tc-DPD was suggestive of transthyretin-related cardiac amyloidosis for nine (18 %) patients, while AL amyloidosis was proven in five patients (10 %) by extracardiac (n = 3, 6 %) or endomyocardial (n = 2, 4 %) biopsies-one patient declined tissue biopsy. Compared to patients with unspecified cardiomyopathy (n = 31), patients with amyloid cardiomyopathy (n = 15 or n = 14/proven) had less hypertension, lower systolic blood pressure and higher NT-pro BNP level. Their electrocardiogram showed lowest QRS voltage and longer QRS duration. Left ventricular (LV) pattern was characterized by a more pronounced LV hypertrophy, a smaller ejection fraction and a decrease of global longitudinal strain associated with an increase of longitudinal strain apical-to-basal ratio. In patients over 65 years, HFpEF is a heterogeneous syndrome with at least a 29 % prevalence of amyloid cardiomyopathy. Combined CMR andTc-DPD are helpful imaging tools for accurate phenotyping of patients amenable to histopathological diagnosis or genetic testing, and should be considered for proper management of this population. Further longitudinal investigations are needed to better clarify these preliminary results.

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“…An elevated jugular vein with diastolic dysfunction noted in echocardiograhy may be attributed to cardiomyopthy due to deposition of light chains albeit tissue diagnosis could not be made. LCDD involving heart can give rise to congestive heart failure to arrhythmias 16. LCDD is associated with monoclonal gammopathies of uncertain significance or multiple myeloma in 17% and 58% of cases, respectively, none of which were present in our case 17…”

Section: Discussionmentioning

confidence: 56%

Portal hypertension related to light chain deposition disease of liver: an enlightening experience

Talukdar¹,

Mukherjee

Khanra

et al. 2013

BMJ Case Reports

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A 55-year-old alcoholic man presented with firm hepatomegaly, ascites and markedly elevated alkaline phosphatase. He had a history of pulmonary tuberculosis. Work-up for malignancy was negative. Histological examination of liver showed extracellular deposition of pink amorphous material which is Congo red stain negative. Deteriorating renal function and nephrotic-range proteinuria were noted. Renal histology showed thickening of the glomerular and tubular basement membranes by non-congophilic deposits along with mesangial expansion. Bone marrow examination revealed patchy areas of pink amorphous deposits which are Congo red stain negative. Immunohistochemical staining of amorphous depositions in liver, kidney and bone marrow were positive for κ light chains. Serum-free light chain assay confirmed markedly elevated free κ-light chain. κ-light chain deposition disease is a systemic disease with universal renal involvement but rarely it presents as chronic cholestatic liver disease with portal hypertension and frequently associated with fatal outcome due to diagnostic delay.

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Cardiac nonamyloidotic immunoglobulin deposition disease

Cited by 38 publications

References 14 publications

Extraosseus Plasmacytoma of the Pharynx with Localized Light Chain Deposition. Case Report

Extraosseus Plasmacytoma of the Pharynx with Localized Light Chain Deposition. Case Report

Pilot study for left ventricular imaging phenotype of patients over 65 years old with heart failure and preserved ejection fraction: the high prevalence of amyloid cardiomyopathy

Portal hypertension related to light chain deposition disease of liver: an enlightening experience

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