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Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report.

Fabbian F, Stabellini N, Sartori S, Tombesi P, Aleotti A, Bergami M, Uggeri S, Galdi A, Molino C, Catizone L - J Med Case Rep (2007)

Bottom Line: Thalidomide and dexamethasone were prescribed, and a peritoneal catheter was inserted.Peritoneal dialysis has now been performed for 15 months without complications.However cardiac involvement is a quite uncommon presentation of LCDD, and this unusual case suggests that the onset of acute arrhythmias associated with restrictive cardiomyopathy and impaired renal function might be related to LCDD.

View Article: PubMed Central - HTML - PubMed

Affiliation: Renal Unit, St, Anna Hospital, Ferrara, Italy. hrfabbia@tin.it.

ABSTRACT

Introduction: Light chain deposition disease (LCDD) can involve the heart and cause severe heart failure. Cardiac involvement is usually described in the advanced stages of the disease. We report the case of a woman in whom restrictive cardiomyopathy due to LCDD presented with paroxysmal atrial fibrillation.

Case presentation: A 55-year-old woman was admitted to our emergency department because of palpitations. In a recent blood test, serum creatinine was 1.4 mg/dl. She was found to have high blood pressure, left ventricular hypertrophy and paroxysmal atrial fibrillation. An ACE-inhibitor was prescribed but her renal function rapidly worsened and she was admitted to our nephrology unit. On admission serum creatinine was 9.4 mg/dl, potassium 6.8 mmol/l, haemoglobin 7.7 g/dl, N-terminal pro-brain natriuretic peptide 29894 pg/ml. A central venous catheter was inserted and haemodialysis was started. She underwent a renal biopsy which showed kappa LCDD. Bone marrow aspiration and bone biopsy demonstrated kappa light chain multiple myeloma. Echocardiographic findings were consistent with restrictive cardiomyopathy. Thalidomide and dexamethasone were prescribed, and a peritoneal catheter was inserted. Peritoneal dialysis has now been performed for 15 months without complications.

Discussion: Despite the predominant tubular deposition of kappa light chain, in our patient the first clinical manifestation of LCDD was cardiac disease manifesting as atrial fibrillation and the correct diagnosis was delayed. The clinical management initially addressed the cardiovascular symptoms without paying sufficient attention to the pre-existing slight increase in our patient's serum creatinine. However cardiac involvement is a quite uncommon presentation of LCDD, and this unusual case suggests that the onset of acute arrhythmias associated with restrictive cardiomyopathy and impaired renal function might be related to LCDD.

No MeSH data available.


Related in: MedlinePlus

Electron microscopy photograph showing nonfibrillar electron-dense deposits in the endothelial side of the glomerular basement membrane.
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Figure 2: Electron microscopy photograph showing nonfibrillar electron-dense deposits in the endothelial side of the glomerular basement membrane.

Mentions: The patient underwent US-guided biopsy of the lower pole of the right kidney, and two specimens were obtained for light and electron microscopy examination. Light microscopy examination showed smooth and continuous deposition of eosinophil material in the tubular basement membrane, mild thickening and stiffness of the glomerular basement membrane, and increase of the mesangial matrix. Congo red stain was negative, but immunofluorescence revealed linear deposits of kappa light chains within the tubular basement membranes. Electron microscopy examination displayed coarse granular electron-dense deposits in the outer surface of the tubular basement membranes (Figure 1), and nonfibrillar electron dense material along the glomerular basement membrane and in the mesangium (Figure 2). Bone marrow aspiration and bone biopsy were performed, and histologic examination of the specimens confirmed the diagnosis of monoclonal immunoglobulin deposition disease associated to kappa light chain multiple myeloma. Treatment with thalidomide 100 mg/day and dexamethasone 40 mg on days 1–4 every 28 days was started, a peritoneal catheter was inserted, and the patient was changed from haemodialysis to peritoneal dialysis. At the time of writing the patient has been dialysing for 15 months and no major complications have been recorded.


Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report.

Fabbian F, Stabellini N, Sartori S, Tombesi P, Aleotti A, Bergami M, Uggeri S, Galdi A, Molino C, Catizone L - J Med Case Rep (2007)

Electron microscopy photograph showing nonfibrillar electron-dense deposits in the endothelial side of the glomerular basement membrane.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2254633&req=5

Figure 2: Electron microscopy photograph showing nonfibrillar electron-dense deposits in the endothelial side of the glomerular basement membrane.
Mentions: The patient underwent US-guided biopsy of the lower pole of the right kidney, and two specimens were obtained for light and electron microscopy examination. Light microscopy examination showed smooth and continuous deposition of eosinophil material in the tubular basement membrane, mild thickening and stiffness of the glomerular basement membrane, and increase of the mesangial matrix. Congo red stain was negative, but immunofluorescence revealed linear deposits of kappa light chains within the tubular basement membranes. Electron microscopy examination displayed coarse granular electron-dense deposits in the outer surface of the tubular basement membranes (Figure 1), and nonfibrillar electron dense material along the glomerular basement membrane and in the mesangium (Figure 2). Bone marrow aspiration and bone biopsy were performed, and histologic examination of the specimens confirmed the diagnosis of monoclonal immunoglobulin deposition disease associated to kappa light chain multiple myeloma. Treatment with thalidomide 100 mg/day and dexamethasone 40 mg on days 1–4 every 28 days was started, a peritoneal catheter was inserted, and the patient was changed from haemodialysis to peritoneal dialysis. At the time of writing the patient has been dialysing for 15 months and no major complications have been recorded.

Bottom Line: Thalidomide and dexamethasone were prescribed, and a peritoneal catheter was inserted.Peritoneal dialysis has now been performed for 15 months without complications.However cardiac involvement is a quite uncommon presentation of LCDD, and this unusual case suggests that the onset of acute arrhythmias associated with restrictive cardiomyopathy and impaired renal function might be related to LCDD.

View Article: PubMed Central - HTML - PubMed

Affiliation: Renal Unit, St, Anna Hospital, Ferrara, Italy. hrfabbia@tin.it.

ABSTRACT

Introduction: Light chain deposition disease (LCDD) can involve the heart and cause severe heart failure. Cardiac involvement is usually described in the advanced stages of the disease. We report the case of a woman in whom restrictive cardiomyopathy due to LCDD presented with paroxysmal atrial fibrillation.

Case presentation: A 55-year-old woman was admitted to our emergency department because of palpitations. In a recent blood test, serum creatinine was 1.4 mg/dl. She was found to have high blood pressure, left ventricular hypertrophy and paroxysmal atrial fibrillation. An ACE-inhibitor was prescribed but her renal function rapidly worsened and she was admitted to our nephrology unit. On admission serum creatinine was 9.4 mg/dl, potassium 6.8 mmol/l, haemoglobin 7.7 g/dl, N-terminal pro-brain natriuretic peptide 29894 pg/ml. A central venous catheter was inserted and haemodialysis was started. She underwent a renal biopsy which showed kappa LCDD. Bone marrow aspiration and bone biopsy demonstrated kappa light chain multiple myeloma. Echocardiographic findings were consistent with restrictive cardiomyopathy. Thalidomide and dexamethasone were prescribed, and a peritoneal catheter was inserted. Peritoneal dialysis has now been performed for 15 months without complications.

Discussion: Despite the predominant tubular deposition of kappa light chain, in our patient the first clinical manifestation of LCDD was cardiac disease manifesting as atrial fibrillation and the correct diagnosis was delayed. The clinical management initially addressed the cardiovascular symptoms without paying sufficient attention to the pre-existing slight increase in our patient's serum creatinine. However cardiac involvement is a quite uncommon presentation of LCDD, and this unusual case suggests that the onset of acute arrhythmias associated with restrictive cardiomyopathy and impaired renal function might be related to LCDD.

No MeSH data available.


Related in: MedlinePlus