AI Article Synopsis
- A 59-year-old woman was diagnosed with primary plasma cell leukemia (PCL), showing symptoms like jaundice, renal dysfunction, anemia, and hypercalcemia, confirmed by specific findings in her blood and bone marrow.
- After initial treatments with steroids showed insufficient results, she was started on bortezomib, leading to a significant decrease in bilirubin levels and noticeable improvement in her health.
- The treatment was adjusted due to side effects, but after pausing, the patient still achieved a "very good partial response," highlighting the potential efficacy of novel treatments for aggressive PCL.
Article Abstract
A 59-year-old woman was admitted to our hospital with jaundice, renal dysfunction, anemia and hypercalcemia. Primary plasma cell leukemia (PCL) was diagnosed based on findings of IgA-λ type M-protein, 22% plasma cells in the bone marrow and 23.1% plasma cells of WBC in the peripheral blood. Because the total bilirubin (T.Bil) level increased even after the administration of prednisolone (PSL), dexamethasone and methylprednisolone, the patient was started on bortezomib (0.7 mg/m(2) on days 1, 4, 8 and 11 for 3 weeks) combined with PSL (40 mg/day). The level of T.Bil decreased and the patient's condition remarkably improved. We then increased the dose of bortezomib to 1.0 mg/m(2) in the second course, but discontinued treatment just after starting the third course because NCI-CTCAE Grade 3 peripheral neuropathy developed. According to the criteria of the International Myeloma Working Group, the response category was VGPR (=very good partial response) at 1 month after pausing treatment. We recommend these novel agents for PCL, which is an aggressive form of extramedullary plasma cell cancer.
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