Clinical analysis of 30 cases of primary plasma cell leukemia
Objective To analyze the clinical characteristics of patients with primary plasma cell leukemia(pPCL)and the effect of combination therapy with proteasome inhibitor(PI),and to investigate the influencing factors of prognosis.Methods The clinical,immunological and cytogenetic characteristics were retrospectively analyzed in 30 pPCL patients(≥5％circulating plasma cells in peripheral blood)who were treated in Nanjing Drum Tower Hospital from March 2014 to June 2023.In 30 patients received first-line PI combination therapy,23 patients received immunomodulators(thalidomide in 7 patients,lenalidomide in 15 patients,and pomalidomide in 1 patient),4 patients received daratumumab,1 patient received cyclophosphamide,and 1 patient received doxorubicin liposome.Four patients received cytotoxic chemotherapy combination regimen,and 4 received venetoclax.Three patients received autologous hematopoietic stem cell transplantation(auto-HSCT)after first-line therapy.The efficacy of the first-line therapy was evaluated once every 2 cycles,and the overall response rate of the first-line therapy was recorded.Five patients received daltolizumab+pomalidomide+dexamethasone after recurrence,among whom 2 patients with progression disease received enatuzumab.The patients were followed up till September 1,2023,to record the total survival and progression-free survival.Univariate and multivariate Cox regression analyses were done to evaluate the influencing factors of death in pPCL patients.Results(1)The main clinical manifestations were anemia in 29 patients,thrombocytopenia in 19,elevated serum lactate dehydrogenase in 14,elevated serum β2-microglobulin in 30;extramedullary lesions in 14;no-expressed CD56 in 17,high-risk cytogenetic abnormalities in 23;t(4;14)in 4,and 1q21 amplification and t(4;14)in 3;revised international staging system stage Ⅲ in 22.(2)After first-line therapy in 30 patients,the efficacy was partial response in 10 patients,very good partial response in 2,complete response in 3,and strict complete response in 3.The overall response rate was 60％.(3)The latest follow-up result was death in 21 patients and survival in 9,and the median progression-free survival was 6 months and median total survival was 8 months.The progression-free survival after strict complete response was 59 and 27 months in two patients receiving auto-HSCT,respectively.(4)The high level of serum lactate dehydrogenase(HR=8.584,95％CI:2.214-33.277,P=0.002),t(4;14)(HR=7.433,95％CI:1.269-43.549,P=0.026),and no use of immunomodulator(HR=0.215,95％CI:0.055-0.850,P=0.028)were the risk factors of death of pPCL patients.Conclusions pPCL is a rare and highly aggressive plasma cell tumor with a high proportion of extramedullary lesions,elevated serum lactate dehydrogenase and β2-microglobulin,usually no-expressed CD56,and a high incidence of high-risk cytogenetic abnormalities.The first-line therapy combined with PI and auto-HSCT can improve the prognosis.pPCL patients with elevated lactate dehydrogenase,t(4;14)and no use of immunomodular have an increased risk of death.
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