Chapter·PathologyGeneral Pathology

AmyloidosisDownloads

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1

A 72-year-old African American man presents with progressive fatigue, difficulty breathing on exertion, and lower extremity swelling for 3 months. The patient was seen at the emergency department 2 times before. The first time was because of back pain, and the second was because of fever and cough. He took medications at the emergency room, but he refused to do further tests recommended to him. He does not smoke or drink alcohol. His family history is irrelevant. His vital signs include a blood pressure of 110/80 mm Hg, temperature of 37.2°C (98.9°F), and regular radial pulse of 90/min. On physical examination, the patient looks pale, and his tongue is enlarged. Jugular veins become distended on inspiration. Pitting ankle edema is present on both sides. Bilateral basal crackles are audible on the chest auscultation. Hepatomegaly is present on abdominal palpation. Chest X-ray shows osteolytic lesions of the ribs. ECG shows low voltage waves and echocardiogram shows a speckled appearance of the myocardium with diastolic dysfunction and normal appearance of the pericardium. Which of the following best describes the mechanism of this patient’s illness?

AThickening of the parietal pericardium with dystrophic calcification

BCalcification of the aortic valve orifice with obstruction of the left ventricular outflow tract

CConcentric hypertrophy of the myocytes with thickening of the interventricular septum

DDiastolic cardiac dysfunction with reciprocal variation in ventricular filling with respiration

EDeposition of an extracellular fibrillar protein that stains positive for Congo red in the myocardium

2

A 65-year-old man comes to the physician because of increasing swelling of the legs and face over the past 2 months. He has a history of diastolic heart dysfunction. The liver and spleen are palpable 4 cm below the costal margin. On physical examination, both lower limbs show significant pitting edema extending above the knees and to the pelvic area. Laboratory studies show: Serum Cholesterol 350 mg/dL (<200 mg/dL) Triglycerides 290 mg/dL (35–160 mg/dL) Calcium 8 mg/dL Albumin 2.8 g/dL Urea nitrogen 54 mg/dL Creatinine 2.5 mg/dL Urine Blood 3+ Protein 4+ RBC 15–17/hpf WBC 1–2/hpf RBC casts Many Echocardiography shows concentrically thickened ventricles with diastolic dysfunction. Skeletal survey shows no osteolytic lesions. Which of the following best explains these findings?

ASmoldering multiple myeloma

BMonoclonal gammopathy of undetermined significance

CWaldenstrom’s macroglobulinemia

DAL amyloidosis

ESymptomatic multiple myeloma

3

A 79-year-old homeless man is brought to the emergency department by ambulance 30 minutes after being found unresponsive by the police. On arrival, he is apneic and there are no palpable pulses. Despite appropriate life-saving measures, he dies. Examination of the heart during autopsy shows normal ventricles with a sigmoid-shaped interventricular septum. A photomicrograph of a section of the heart obtained at autopsy is shown. Which of the following is the most likely underlying cause for the structure indicated by the arrow?

AAccumulation of iron granules

BClumping of defective mitochondria

CDeposition of wild-type transthyretin

DAggregation of alpha-synuclein

EOxidation of phospholipid molecules

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