A 62-year-old man presents with bone pain and fatigue. Serum protein electrophoresis shows an M-spike, and bone marrow biopsy reveals 45% plasma cells. FISH cytogenetics shows the pattern marked **B** in the diagram — multiple gains with trisomies of chromosomes 3, 5, 7, 9, 11, 15, 19, and 21. Total chromosome count is 52. According to current risk stratification (mSMART/R-ISS), this cytogenetic finding places the patient in which risk category?
A. Standard-risk only if combined with t(11;14); otherwise this pattern is considered intermediate-risk
B. High-risk; the total chromosome count of 52 exceeds the threshold for standard-risk disease
C. Standard-risk; hyperdiploid myeloma typically has favorable prognosis with modern therapy
D. High-risk; trisomies of odd chromosomes are associated with aggressive disease and early relapse
Explanation
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Why Standard-risk; hyperdiploid myeloma typically has favorable prognosis with modern therapy is right
The pattern marked B — hyperdiploid myeloma with trisomies of odd-numbered chromosomes (3, 5, 7, 9, 11, 15, 19, 21) and a total chromosome count of 48–74 — is classified as STANDARD-RISK disease according to mSMART and R-ISS criteria (Rajkumar, Mayo Clin Proc 2022; IMWG 2014). Hyperdiploid MM accounts for 50–55% of all MM cases and is characterized by multiple chromosomal gains derived from post-germinal-center plasma cells, typically WITHOUT primary IGH translocations. This cytogenetic subtype is associated with a lower proliferative index and a 5-year overall survival of approximately 70–75% with modern therapy (proteasome inhibitors, immunomodulatory drugs, and autologous stem cell transplantation).
Why each distractor is wrong
High-risk; trisomies of odd chromosomes are associated with aggressive disease and early relapse: This directly contradicts the IMWG risk stratification. Trisomies of odd chromosomes define FAVORABLE/STANDARD-RISK hyperdiploid MM, not high-risk disease. High-risk cytogenetics include t(4;14), t(14;16), t(14;20), del(17p), and 1q gain — not hyperdiploid trisomies.
High-risk; the total chromosome count of 52 exceeds the threshold for standard-risk disease: Incorrect. Hyperdiploid MM is defined as 48–74 total chromosomes. A count of 52 falls squarely within the hyperdiploid range and is STANDARD-RISK, not high-risk. The threshold is not a risk determinant; the presence of trisomies of ODD chromosomes is.
Standard-risk only if combined with t(11;14); otherwise this pattern is considered intermediate-risk: Hyperdiploid MM with trisomies of odd chromosomes is STANDARD-RISK on its own; t(11;14) is a separate standard-risk translocation. There is no "intermediate-risk" category in mSMART/R-ISS — only standard-risk and high-risk.
High-YieldNEET PG
Hyperdiploid MM (48–74 chromosomes with trisomies of odd chromosomes 3, 5, 7, 9, 11, 15, 19, 21) = STANDARD-RISK; high-risk = t(4;14), t(14;16), del(17p), 1q gain.
