A 55-year-old male presented with easy bruising and recurrent nose bleeds.
He had mild anemia with Hb of 7.2 mM, and extensive laboratory work-up showed the presence of an IgM-kappa M-protein of 41 g/l (normal levels of the free light chains), as well as a coagulation tests compatible with acquired von Willebrand’s disease (low VWF antigen, even lower VWF activity, low FVIII, decrease in HMW VWF multimers, and presence of an inhibitory antibody in neutralization assays). There were no signs of hyperviscosity.
Whole body CT showed no abnormalities. Bone marrow examination revealed 25% clonal plasma cells. Cytogenetic evaluation showed hyperdiploidy in the clonal plasma cells, and molecular tests showed no MYD88 mutation. There were no other laboratory abnormalities.
Options
- Start treatment for Waldenstrom’s macroglobulinemia: start ibrutinib therapy
- Start treatment for Waldenstrom’s macroglobulinemia: start DRC therap
- Start treatment for myleloma: start induction followed by ASCT plus lenalidomide maintenance
- Start treatment for myleloma: start daratumumab-based therapy without ASCT
Answer: 3
Patient has a clonal IgM-producing plasma cell proliferation, whereby the hyperdiploidy detected by cytogenetic analysis, is compatible with a diagnosis of a plasma cell dyscrasia. In the absence of SLiM/CRAB symptoms, we concluded that the patient is suffering from smoldering multiple myeloma with acquired von Willebrand’s disease. Given his recurrent bleeds, we started treatment with a daratumumab-VTD (D-VTD) followed by transplant, and then D-VTD consolidation and lenalidomide maintenance, aiming at achieving a deep/prolonged remission.
