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A Case of AL Amyloidosis with Negative Abdominal Fat Pad Biopsy in the Setting of High Clinical Suspicion
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A3399 - A Case of AL Amyloidosis with Negative Abdominal Fat Pad Biopsy in the Setting of High Clinical Suspicion
Author Block: M. Khan1, F. M. Khateeb2, Z. Khan1, A. Moustafa1, D. Hernandez2; 1Internal Medicine, University of Toledo Medical Center, Toledo, OH, United States, 2Pulmonary/Critical Care, University of Toledo Medical Center, Toledo, OH, United States.
Introduction: The amyloidoses are rare group of diseases characterized by extracellular deposition of insoluble proteins either systemically or in specific organs. Although systemic amyloidosis may be suspected clinically, diagnosis can be established only from histopathological examination by tissue biopsy. Abdominal fat pad biopsy is preferred method of obtaining of tissue sample in suspected cases with good sensitivity and excellent specificity for diagnosis of systemic amyloidosis. We present a case in which systemic amyloidosis was suspected but biopsy from abdominal fat pad was negative for Congo stain.
Case Report: 64year old Caucasian female who initially presented to the hospital with chest pain and dyspnea. Electrocardiogram showed low voltage QRS complexes. An echocardiogram showed diffuse left ventricular wall hypertrophy with grade 2 diastolic dysfunction. Cardiac catheterization was performed which showed nonobstructive coronary arteries. She had stage II chronic kidney disease (baseline creatinine of 1.2 mg/dL) and a urinalysis done in hospital showed nephrotic range proteinuria. Further workup showed elevated kappa and lambda free light chains in serum and urine. Cardiac MRI was obtained which showed septal predominant left ventricular hypertrophy. At this time there was high clinical suspicion of systemic amyloidosis with cardiac and renal involvement and abdominal fat pad biopsy was obtained. Abdominal fat pad biopsy however was negative for Congo red and amyloid SAB stain. Over the course of several days patient’s condition declined precipitously with rapid worsening of renal functions requiring hemodialysis, development of pneumonia with sepsis and an episode of bradycardia and asystole leading to cardiac arrest. Cardiopulmonary resuscitation was performed and return of spontaneous circulation was achieved. Patient was transferred to intensive care unit for further care. However patient continue to deteriorate despite aggressive medical management. After discussing with the family care was with withdrawn and patient passed away. Autopsy was obtained post mortem and showed lambda light chain systemic amyloidosis involving multiple organs.
Discussion: Abdominal fat pad aspiration is preferred method for detecting amyloidosis. However in cases of high clinical suspicion where fat pad biopsy is negative, organ specific biopsies should be obtained to successfully rule in or rule out systemic amyloidosis.
Author Block: M. Khan1, F. M. Khateeb2, Z. Khan1, A. Moustafa1, D. Hernandez2; 1Internal Medicine, University of Toledo Medical Center, Toledo, OH, United States, 2Pulmonary/Critical Care, University of Toledo Medical Center, Toledo, OH, United States.
Introduction: The amyloidoses are rare group of diseases characterized by extracellular deposition of insoluble proteins either systemically or in specific organs. Although systemic amyloidosis may be suspected clinically, diagnosis can be established only from histopathological examination by tissue biopsy. Abdominal fat pad biopsy is preferred method of obtaining of tissue sample in suspected cases with good sensitivity and excellent specificity for diagnosis of systemic amyloidosis. We present a case in which systemic amyloidosis was suspected but biopsy from abdominal fat pad was negative for Congo stain.
Case Report: 64year old Caucasian female who initially presented to the hospital with chest pain and dyspnea. Electrocardiogram showed low voltage QRS complexes. An echocardiogram showed diffuse left ventricular wall hypertrophy with grade 2 diastolic dysfunction. Cardiac catheterization was performed which showed nonobstructive coronary arteries. She had stage II chronic kidney disease (baseline creatinine of 1.2 mg/dL) and a urinalysis done in hospital showed nephrotic range proteinuria. Further workup showed elevated kappa and lambda free light chains in serum and urine. Cardiac MRI was obtained which showed septal predominant left ventricular hypertrophy. At this time there was high clinical suspicion of systemic amyloidosis with cardiac and renal involvement and abdominal fat pad biopsy was obtained. Abdominal fat pad biopsy however was negative for Congo red and amyloid SAB stain. Over the course of several days patient’s condition declined precipitously with rapid worsening of renal functions requiring hemodialysis, development of pneumonia with sepsis and an episode of bradycardia and asystole leading to cardiac arrest. Cardiopulmonary resuscitation was performed and return of spontaneous circulation was achieved. Patient was transferred to intensive care unit for further care. However patient continue to deteriorate despite aggressive medical management. After discussing with the family care was with withdrawn and patient passed away. Autopsy was obtained post mortem and showed lambda light chain systemic amyloidosis involving multiple organs.
Discussion: Abdominal fat pad aspiration is preferred method for detecting amyloidosis. However in cases of high clinical suspicion where fat pad biopsy is negative, organ specific biopsies should be obtained to successfully rule in or rule out systemic amyloidosis.
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