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Primary plasma cell leukemia (pPCL) is a rare and aggressive plasma cell disorder with distinct clinical and biological features.Thanks to the application of novel agents such as proteasome inhibitors (e.g.bortezomib) and immunomodulatory drugs (e.g.thalidomide and lenalidomide),along with stem cell transplantation,the outcome of pPCL has been improved a lot.Despite therapeutic advances,the current understanding of pPCL biology is still limited due to the lack of availability of clinical samples.This review provides the recent progress of the diagnostic and therapeutic options in pPCL.
RESUMO
Objective To investigate the clinical characteristics of plasma cell malignancies with t(11;14) and the effect of t(11;14) on prognosis. Methods A cohort of 380 newly diagnosed patients with plasma cell malignancies were analyzed,including 146 females and 234 males.There were 370 cases of newly diagnosed multiple myeloma (NDMM), as well as 10 cases of primary plasma cell leukemia (PCL). The relationship between the categorical variables was evaluated by using the bilateral Fisher exact probability test, with 95 % confidence interval. Results Of 370 NDMM cases, t(11;14) was detected in 101 cases (27.3 %). Of 10 PCL cases, 8 cases displayed t(11;14). The detection rate of t(11;14) was significantly higher in IgD, IgM and non-secreting MM [50.9 % (27/53)] than that in IgA MM [21.6 % (16/78)] and IgG [28.4 % (52/183)] (both P= 0.002). The rate of CD56+in t(11;14) positive group was lower than that in t(11;14) negative group [51.6 % (48/93) vs. 72.0 % (167/232), P= 0.001], and the rate of CD117+was also significantly decreased [23.7 % (22/93) vs. 37.7 % (87/231), P= 0.019]. There were 86 cases of non-t(11;14) IgH rearrangement in 269 cases of NDMM without t(11;14), which mainly were t(4;14) or t(14;16). The detection rate of high risk MM was only 11.9 %(12/101)in t(11;14)positive group,while that rate was 27.5 % (74/269) in t(11;14) negative group, the difference was statistically significant (P = 0.001). Conclusion MM with t(11;14)displays distinct biological,clinical and laboratory features,it is a heterogeneous disease.
RESUMO
Objective To investigate the clinical characteristics of plasma cell malignancies with t(11;14) and the effect of t(11;14) on prognosis. Methods A cohort of 380 newly diagnosed patients with plasma cell malignancies were analyzed,including 146 females and 234 males.There were 370 cases of newly diagnosed multiple myeloma (NDMM), as well as 10 cases of primary plasma cell leukemia (PCL). The relationship between the categorical variables was evaluated by using the bilateral Fisher exact probability test, with 95 % confidence interval. Results Of 370 NDMM cases, t(11;14) was detected in 101 cases (27.3 %). Of 10 PCL cases, 8 cases displayed t(11;14). The detection rate of t(11;14) was significantly higher in IgD, IgM and non-secreting MM [50.9 % (27/53)] than that in IgA MM [21.6 % (16/78)] and IgG [28.4 % (52/183)] (both P= 0.002). The rate of CD56+in t(11;14) positive group was lower than that in t(11;14) negative group [51.6 % (48/93) vs. 72.0 % (167/232), P= 0.001], and the rate of CD117+was also significantly decreased [23.7 % (22/93) vs. 37.7 % (87/231), P= 0.019]. There were 86 cases of non-t(11;14) IgH rearrangement in 269 cases of NDMM without t(11;14), which mainly were t(4;14) or t(14;16). The detection rate of high risk MM was only 11.9 %(12/101)in t(11;14)positive group,while that rate was 27.5 % (74/269) in t(11;14) negative group, the difference was statistically significant (P = 0.001). Conclusion MM with t(11;14)displays distinct biological,clinical and laboratory features,it is a heterogeneous disease.