Amyloidosis types — MCQs

A previously healthy 82-year-old man dies in a motor vehicle collision. At autopsy, the heart shows slight ventricular thickening. There are abnormal, insoluble aggregations of protein filaments in beta-pleated linear sheets in the ventricular walls and, to a lesser degree, in the atria and lungs. No other organs show this abnormality. Bone marrow examination shows no plasma cell dyscrasia. The abnormal protein aggregations are most likely composed of which of the following?

A 48-year-old woman comes to the physician because of progressively worsening dyspnea on exertion and fatigue for the past 2 months. She had Hodgkin lymphoma as an adolescent, which was treated successfully with chemotherapy and radiation. Her father died from complications related to amyloidosis. She does not smoke or drink alcohol. Her temperature is 36.7°C (98°F), pulse is 124/min, respirations are 20/min, and blood pressure is 98/60 mm Hg. Cardiac examination shows no murmurs. Coarse crackles are heard at the lung bases bilaterally. An ECG shows an irregularly irregular rhythm with absent P waves. An x-ray of the chest shows globular enlargement of the cardiac shadow with prominent hila and bilateral fluffy infiltrates. Transthoracic echocardiography shows a dilated left ventricle with an ejection fraction of 40%. Which of the following is the most likely cause of this patient's condition?

A 63-year-old man presents to the emergency department with periorbital swelling. He states that he was gardening, came inside, looked in the mirror, and then noticed his eyelids were swollen. He denies pain, pruritus, or visual disturbances. He states that he was drinking “a lot of water" to prevent dehydration, because it was hot outside this morning. His medical history is significant for rheumatoid arthritis. He takes methotrexate and acetaminophen as needed. The patient’s temperature is 98°F (36.7°C), blood pressure is 168/108 mmHg, and pulse is 75/min. Physical examination is notable for periorbital edema, hepatomegaly, and bilateral 1+ pitting lower extremity edema. Labs and a urinalysis are obtained, as shown below: Leukocyte count: 11,000/mm^3 Hemoglobin: 14 g/dL Serum: Na: 138 mEq/L K+: 4.3 mEq/L Cl-: 104 mEq/L HCO3-: 25 mEq/L Urea nitrogen: 26 mg/dL Creatinine: 1.4 mg/dL Glucose: 85 mg/dL Aspartate aminotransferase (AST, GOT): 15 U/L Alanine aminotransferase (ALT, GPT): 19 U/L Albumin: 2.0 g/dL Urine: Protein: 150 mg/dL Creatinine: 35 mg/dL An abdominal ultrasound reveals an enlarged liver with heterogeneous echogenicity and enlarged kidneys with increased echogenicity in the renal parenchyma. A biopsy of the kidney is obtained. Which of the following biopsy findings is associated with the patient’s most likely diagnosis?

A 42-year-old man with systolic heart failure secondary to amyloidosis undergoes heart transplantation. The donor heart is obtained from a 17-year-old boy who died in a motor vehicle collision. Examination of the donor heart during the procedure shows a flat, yellow-white discoloration with an irregular border on the luminal surface of the aorta. A biopsy of this lesion is most likely to show which of the following?

A 55-year-old man comes to the physician because of a 3-day history of decreased urine output, progressively worsening bilateral pedal edema, and fatigue. He has a 4-month history of persistent lower back pain. He has hypercholesterolemia and stable angina pectoris. Current medications include atorvastatin, aspirin, and ibuprofen. His pulse is 80/min, respirations are 16/min, and blood pressure is 150/100 mm Hg. Examination shows periorbital and pedal edema and pallor. There is tenderness of the lumbar spinal vertebrae. Straight leg raise test is negative. The remainder of the examination shows no abnormalities. Laboratory studies show: Hemoglobin 8.9 g/dl Serum Urea nitrogen 20 mg/dl Creatinine 2.4 mg/dl Calcium 11.2 mg/dl Alkaline phosphatase 140 U/L X-ray of the spine shows diffuse osteopenia and multiple lytic lesions. Which of the following is most likely to confirm the diagnosis?

A 36-year-old man comes to the physician for a 4-week history of swollen legs. He has difficulty putting on socks because of the swelling. Two years ago, he was diagnosed with sleep apnea. He takes no medications. He emigrated from Guatemala with his family when he was a child. He is 171 cm (5 ft 6 in) tall and weighs 115 kg (253 lb); BMI is 39 kg/m2. His pulse is 91/min and blood pressure is 135/82 mm Hg. Examination shows periorbital and bilateral lower extremity edema. Serum Albumin 3.1 g/dL Total cholesterol 312 mg/dL Urine Blood negative Protein +4 RBC 1-2/hpf RBC cast negative Fatty casts numerous A renal biopsy is obtained. Which of the following is most likely to be seen under light microscopy of the patient's renal biopsy specimen?

A 7-year-old girl is brought to her pediatrician by her mother because of puffiness under both eyes in the morning. The mother reports that the child has just recovered from a seasonal influenza infection a few days ago. Vital signs include: temperature 37°C (98.6°F), blood pressure 100/67 mm Hg, and pulse 95/min. On examination, there is facial edema and bilateral 2+ pitting edema over the legs. Laboratory results are shown: Serum albumin 2.1 g/dL Serum triglycerides 200 mg/dL Serum cholesterol 250 mg/dL Urine dipstick 4+ protein Which of the following casts are more likely to be present in this patient’s urine?

A 52-year-old man presents with 2 months of diarrhea, abdominal pain, and fatigue. He reports a weight loss of 4 kg (8 lb). He also says his joints have been hurting recently, as well. Past medical history is unremarkable. Review of systems is significant for problems with concentration and memory. Physical examination is unremarkable. A GI endoscopy is performed with a biopsy of the small bowel. Which of the following histologic finding would most likely be seen in this patient?

A 60-year-old woman comes to the physician because of lower back pain, generalized weakness, and weight loss that has occurred over the past 6 weeks. She also says that her urine has appeared foamy recently. Physical examination shows focal midline tenderness of the lumbar spine and conjunctival pallor. Her temperature is 100.5°F (38°C). A photomicrograph of a bone marrow biopsy specimen is shown. Further evaluation of this patient is most likely to show which of the following findings?

A 62-year-old man comes to the physician because of fatigue and decreased urine output for 2 weeks. He has not been to the physician for many years and takes no medications. Serum studies show a urea nitrogen concentration of 42 mg/dL and a creatinine concentration of 2.3 mg/dL. Urinalysis shows heavy proteinuria. A photomicrograph of a section of a kidney biopsy specimen is shown. Which of the following is the most likely underlying cause of this patient's symptoms?