Secondary Plasma Cell Leukemia:one Case Report And Literature Review

Objective To investigate the causes of multiple myeloma(MM)following secondary plasma cell leukemia(sPCL)and to improve the understanding of sPCL.Methods:A retrospective study was performed on a case of MM patient who was followed sPCL,we analyzed the clinical feature,laboratory examination and the treatment of sPCL.For the diagnosis of sPCL,refer to references:more than 20%plasma cells in peripheral blood and an absolute plasma cell count greater than 2×10~9.Results:This case is a 62-year-old female with bone pain and anemia as the main initial clinical manifestations.She was diagnosed as high risk multiple myeloma with the proportion of bone marrow plasma cells was 37.5%,with TP53 locus deletion positive(18%)and 1q21 locus amplification positive(8%).Plasma cell leukemia(PCL)developed after 2 courses of BCD regimen with the proportion of bone marrow and peripheral blood plasma cells were60.5%and 21%,respectively.The immunophenotypes of MM cells are CD138+,CD38+,CD56+and CD20-,and CyclinD1+is newly added after PCL.New cytogenetic changes t(X;1)(q12;q43),t(11;14)(q14;q32),in addition to massive pleural effusion was found on both sides.After two courses of VD-PACE chemotherapy,complete remission(CR)was achieved.Conclusion:sPCL is a rare,but very aggressive,plasma cell dyscrasia,representing a distinct clinico pathological entity as compared to MM,with peculiar biological and clinical features.Genetic manifestations of high risk,especially"double hit","triple hit"and newly developed t(11;14)high-risk MM with chromosomal abnormalities are more likely to be converted to PCL.However,once pleural effusion occurs in such MM patients,the possibility of sPCL should be warned.Intensive chemotherapy with bortezomib is still an effective therapeutic method.