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Self-Assessment Questions

Hematology

Primary Systemic Amyloidosis: Review Questions

Jeffrey A. Zonder, MD

Dr. Zonder is an assistant professor of medicine, Division of Hematology-Oncology,
Karmanos Cancer Institute, Wayne State University School of Medicine, Detroit, MI.



Choose the single best answer for each question.

All of the questions refer to the following case.

At his first visit with a hematologist, a 68-year-old Caucasian man states that he has been diagnosed with amyloidosis, but he is unsure of which type. The diagnosis was made after he developed peripheral sensory neuropathy, tongue enlargement, and intestinal malabsorption. He also reports recent onset of dyspnea and orthopnea.

1. Which of the following would be the best test to confirm whether this patient has primary systemic (AL) amyloidosis?

  1. Congo red staining of fat pad aspirate
  2. Serum and urine protein electrophoresis with immunofixation
  3. Echocardiography
  4. κ/λ Immunostaining of tissue amyloid deposits
  5. Transthyretin mutation analysis
Click here to compare your answer.


2. After the diagnosis of AL-amyloidosis is confirmed, the patient undergoes an echocardiogram, which reveals a thickened ventricular septal wall, impaired diastolic relaxation, and a left ventricular ejection fraction of 35%. Which of the following would be the best choice of initial therapy?

  1. 5-Drug combination chemotherapy regimen (VBMCP)
  2. Melphalan and prednisone (MP)
  3. Colchicine
  4. MP and colchicine
  5. High-dose thalidomide
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3. After initiating treatment, the patient returns for routine follow-up and states that several acquaintances from a local amyloidosis support group were treated with high-dose melphalan and autologous stem cell transplant (ASCT). He asks whether this therapy might be appropriate for him. Which of the following statements regarding ASCT for amyloidosis is correct?
  1. There have been no randomized trials demonstrating improved survival following ASCT versus conventional nontransplant therapy
  2. Treatment-related mortality is similar to that in multiple myeloma
  3. This patientŐs amyloid-related cardiac dysfunction does not have prognostic significance in relation to ASCT as long as symptoms are well-controlled with medications
  4. Prior chemotherapy precludes further consideration of ASCT because stem cell collection will be problematic
  5. ASCT cannot be considered in this case because the patient is older than 65 years
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4. The patient responds to initial therapy, but 8 months later develops progressive weight gain, lower extremity edema, anasarca, ascites, and hypotension. On examination, no jugular venous distention is observed, but the liver is enlarged. What is the most likely cause of these findings?

  1. Hepatic amyloid deposition with resultant portal hypertension
  2. Renal amyloid deposition with resultant nephrotic syndrome
  3. Splenic amyloid deposition with capsular rupture and bleeding
  4. Cardiac amyloid deposition with resultant right-sided heart failure
  5. High-dose aspirin alone
Vascular wall amyloid deposition with resultant deep vein thrombosis Click here to compare your answer.
 

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Updated 4/20/07 • kkj