浆母细胞性骨髓瘤的临床表现与治疗——一项多中心回顾性分析

To report the clinical manifestations, laboratory tests, pathological features, diagnosis and treatment process and outcome of six patients with plasmablastic myeloma (PBM), and the clinical features and diagnosis and treatment methods of PBM were discussed in combination with literature.

The present study is a multicenter case series involving a small sample of six patients (n = 6). Clinical data of patients, such as age of onset, sex, pathological type, cytogenetic characteristics, disease stage, extramedullary disease were analyzed, and the efficacy evaluation and outcome after treatment were observed.

Among the 6 patients with PBM, 2 male and 4 female. The median age of the 6 patients was 63 (range 46-68) years. Three cases were newly diagnosed, three cases were found at the time of recurrence. The Ki-67 proliferation index of 5 cases was above 65%. Six cases all developed extramedullary infiltrates. Five cases were positive for 1q21 amplification. Four cases were negative for CD56 and one case was partially positive. Five patients died after multiple cycles of chemotherapy, and one patient is under treatment.

PBM has a low incidence, rapid cell proliferation, high tumor burden, multiple high-risk genetic abnormalities, rapid progression and poor prognosis. PBM frequently affects extramedullary sites. There is currently no standard treatment for PBM. CAR-T therapy may be a new option. Allogeneic hematopoietic stem cell transplantation is also the direction of exploration.