case_images: PMC5485300_01
case_images: Laboratory response of AVWS after treatment of lymphoplasmacytic lymphoma with carfilzomib, rituximab, and dexamethasone. Arrows indicate cycles of treatment. Treatment resulted in normalization of VWF antigen level (VWF:Ag), VWF activity (VWF:RCo), and factor VIII activity assay (FVIII activity). Normal values are shown in parentheses.
case_images: CRIHEM2017-9862620.001.jpg
case_images: PMC5485300_01_CRIHEM2017-9862620.001.jpg
case_images: fig1
case_images: Serial IgM concentrations are shown in Figure 1. In the past, she had iron deficiency anemia which had responded to oral iron replacement (see Figure 1 for serial ferritin and hemoglobin levels). Serum creatinine is shown in Figure 1. AVWS was the monoclonal gammopathy. Although she had a normal bone marrow biopsy at the time the MGUS was diagnosed and her M-protein concentration had remained stable over time (Figure 1), her bleeding symptoms were becoming more frequent and there was a possibility of sampling error of the first bone marrow biopsy. Figure 1 shows the timing of her treatment and the concomitant changes in VWF:Ag, VWF:RCo, and FVIII activity.
case_images: Lymphoplasmacytic lymphoma, H&E (a). Plasma cells with Dutcher bodies (b). Lymphocytes and plasma cells with Dutcher bodies and Russell bodies (c). Kappa light chain stain (d).
case_images: CRIHEM2017-9862620.002.jpg
case_images: PMC5485300_01_CRIHEM2017-9862620.002.jpg
case_images: fig2
case_images: Bone marrow biopsy now showed 70% involvement by lymphoplasmacytic lymphoma (LPL) (Figure 2).