FRCPath Haem Part 1 MCQs-Oncology 481
A 61-year-old man is diagnosed with systemic primary AL amyloidosis.
Investigations show:
Bone marrow plasma cells: 8%
Serum free light chains: markedly abnormal
dFLC: 185 mg/L
Serum paraprotein: 3 g/L
NT-proBNP: moderately elevated
Troponin-T: mildly elevated
Echocardiogram: increased ventricular wall thickness with preserved ejection fraction
eGFR: 68 mL/min/1.73 m²
No significant peripheral neuropathy
No significant gastrointestinal or respiratory involvement
He is otherwise fit and has no major comorbidities.
Which of the following is the MOST appropriate initial treatment?
A. High-dose melphalan followed immediately by autologous stem-cell transplantation
B. Daratumumab + bortezomib + cyclophosphamide + dexamethasone (Dara-VCD)
C. Lenalidomide + dexamethasone
D. Melphalan + dexamethasone alone
E. Pomalidomide + dexamethasone
Correct answer: B. Daratumumab + bortezomib + cyclophosphamide + dexamethasone (Dara-VCD)
Explanation
Dara-VCD is the standard first-line approach for most newly diagnosed systemic AL amyloidosis patients requiring treatment.
The rationale is:
AL amyloidosis requires rapid suppression of the pathogenic plasma-cell clone.
Organ improvement generally lags behind the haematological response, sometimes by many months.
Therefore, achieving a rapid deep clonal response is crucial.
Daratumumab + VCD has become the established first-line regimen following the ANDROMEDA trial.
NICE approved daratumumab with VCD for newly diagnosed systemic AL amyloidosis in the UK.
Why not autologous transplant?
Although high-dose melphalan with autologous stem-cell transplantation can produce very deep responses, it is reserved for carefully selected, transplant-eligible patients.
The exam trap is that this patient…