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1 FastTest You ve read the book now test yourself To ensure you have learned the key points that will improve your patient care, read the authors questions below. Please refer back to relevant sections of the book to address any areas of uncertainty.

2 1 Epidemiology and etiology 1. Which of the following statements are true? a) Multiple myeloma is the second most common hematologic malignancy in Europe. b) The incidence of myeloma increases with age. c) There is no difference in incidence of myeloma between the sexes. d) White populations are at the highest risk of developing myeloma. e) There is a possible association between myeloma risk and BRCA1 and BRCA2 mutations. f) There is strong evidence for viral infection as an acquired cause of myeloma.

3 2 Predisposing conditions associated with myeloma 2. Which of the following statements are true? a) There are two types of monoclonal gammopathy of undetermined significance (MGUS), depending on the monoclonal immunoglobulin (Ig) (M protein) that is present. b) Smoldering multiple myeloma (SMM) is asymptomatic. c) A serum M protein level of more than 3.0 g/dl suggests a diagnosis of MGUS. d) Detection of more than 10% bone marrow (BM) plasma cells indicates MGUS. e) SMM is associated with end organ damage.

4 3. Which of the following clinical markers are associated with increased risk of progression from MGUS to myeloma? a) Serum level of Ig more than 1.5 g/dl (> 15 g/l). b) Abnormal free light chain (FLC) ratio (< 0.26 or > 1.65). c) Non-IgG isotype. d) Abnormal plasma cells (apc) of more than 95%. e) DNA aneuploidy for MGUS.

5 3 Pathophysiology of myeloma and MGUS 4. Which of the following statements are true? a) The clonal plasma cells in MGUS have a different phenotype to that of healthy plasma cells, and a higher proliferation rate. b) The clonal plasma cells in MGUS have a different phenotype to those found in myeloma. c) Myeloma is characterized by a clonal population of tumor cells that secrete large amounts of antibodies. d) The survival of myeloma cells in the microenvironment requires a numer of cytokines and trophic factors including IL-6 and TNFα. e) Each Ig molecule in plasma cells has both a kappa (κ) and lambda (λ) light chain; elevations of these FLCs in the blood indicate a monoclonal gammopathy.

6 4 Diagnosis, staging and monitoring of myeloma 5. Early diagnosis of myeloma improves clinical outcomes. Which of the following statements are true? a) Patients with myeloma often present with fever, night sweats and weight loss. b) Anemia is a common presenting feature of myeloma. c) A complete biochemistry profile often reveals hypocalcemia in patients with myeloma. d) A peripheral blood smear will reveal aggregation or stacking of red blood cells (Rouleaux formation). e) Renal function is abnormal in up to one-third of patients with myeloma.

7 6. Which of the following statements are true? a) Using the International Staging System (ISS) for myeloma, stage III is characterized by a serum β2-microglobulin level of mg/l with any albumin level. b) Dual-energy X-ray absorptiometry (DEXA) is the technique of choice at diagnosis for identifying areas of bone damage in patients with myeloma. c) Ig heavy chain gene translocations are associated with adverse outcomes in myeloma. d) A stringent complete response (scr) is defined as a normal FLC ratio and the absence of clonal cells in bone marrow. e) Biochemical relapse is defined as a rise in serum/urine clonal protein without clinical symptoms.

8 5 Genetics and myeloma 7. Identification of genetic differences between patients with myeloma helps to tailor the treatment approach. Which of the following statements are true? a) The presence of any cytogenetic abnormality on routine karyotyping in patients with myeloma is associated with high-risk disease. b) Hyperdiploidy (increased numbers of odd-numbered chromosomes) is associated with adverse outcomes. c) Del 13q, when present in isolation, is associated with adverse outcomes. d) The presence of genetic aberrations affects the choice of induction therapy. e) Initial genome sequencing studies in patients with myeloma found that mutations affecting the NRAS and KRAS genes were most common.

9 6 Induction therapy for newly diagnosed myeloma 8. Who is eligible for induction therapy? Any patient with: a) symptomatic myeloma (end organ damage) b) abnormal plasma cells of 10% or more c) an sflc ratio of more than 100 d) more than one focal bone lesion on MRI e) smoldering myeloma.

10 9. Which of the following statements are true? a) Induction with a two-drug regimen is standard practice for most transplant-eligible patients. b) Autologous stem cell transplantation should be delayed in patients who achieve complete remission after induction therapy. c) A second transplant following relapse is becoming more common. d) No drug combination has been able to improve on melphalan prednisone (MP) for the treatment of transplant-ineligible patients. e) Regimens that include lenalidomide or bortezomib are the current treatment of choice for high-risk patients.

11 7 Transplantation and myeloma 10. Which of the following statements are true? a) High-dose therapy and autologous transplant confer significant survival benefits, even after a major response is achieved with a modern induction regimen. b) A number of studies have reported benefits with high-dose therapy in older patients. c) Lower doses of melphalan (140 mg/m 2 ) should be used in older patients. d) Randomized clinical trials have shown significant survival benefit in high-risk patients who received allogeneic stem cell transplantation.

12 8 Relapsed and refractory myeloma 11. Progressive disease (relapse) is defined as: a) at least a 50% increase from nadir in serum or urine paraprotein levels b) an absolute increase of serum or urine paraprotein levels of 0.5 g/ dl or more c) at least a 25% elevation from nadir of sflcs d) an abnormal FLC ratio e) a 10% or more increase in BM plasma cells.

13 9 Bone disease and renal complications in myeloma 12. Which of the following statements are true? a) Up to 80% of patients present with bone lesions on skeletal radiography at diagnosis. b) Patients with myeloma with bone disease should receive regular bisphosphonate therapy. c) Fewer than 10% of patients present with renal dysfunction at diagnosis. d) A kidney biospy is often required to determine the type of renal injury. e) Myeloma with renal failure is an emergency, and treatment should be started as soon as possible.

14 10 AL amyloidosis 13. Which of the following statements are true? a) AL amyloidosis is the most common form of systemic amyloidosis b) The presence of a paraprotein in the serum or urine confirms diagnosis c) Systemic AL amyloidosis is staged by the extent of cardiac involvement and sflcs d) High-dose melphalan and autologous stem cell transplantation is the treatment of choice for young fit patients e) Radiotherapy is first-line treatment for patients who are not eligible for transplantation

15 11 Rare plasma cell dyscrasias 14. Which of the following statements are true? a) Solitary medullary or extramedullary plasmacytoma is a curable disorder b) A high level of sflcs is a risk factor for early disease progression in patients with plasmacytoma c) Patients with Waldenström s macroglobulinemia (WM) tend to have a high symptom burden and require intensive supportive care d) Rituximab-based combination therapy is suitable for both newly diagnosed and relapsed good-performance-status patients with WM e) Anemia is the main presenting symptom in patients with POEMS syndrome

16 11 Supportive care 15. Which of the following statements are true? a) Up to three-quarters of patients with myeloma present with anemia. b) Infection is one of the commonest causes of early deaths in patients with myeloma. c) Thalidomide and bortezomib can both cause neuropathy in patients with myeloma. d) Bleeding is rare in myeloma. e) None of the above.

17 Thank you for taking this FastTest. There is no final score or certificate, but by reinforcing your understanding you have enhanced your clinical skills and practice. If you found the book useful, please spread the word about Fast Facts to your colleagues, students and patients.

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