Plasma Cell Leukamia:report Of 6 Cases And Review Of Literature

Objective: To summarize the clinical data of patients with plasma cell leukemia(PCL),provide a theoretical basis for improving the level of diagnosis and treatment.Methods: Six patients diagnosed with plasma cell leukemia in the Hebei General Hospital from June 2013 to June 2020 were reported.The clinical features and laboratory characteristic were investigated along with a review of the literature.Results:1.In this group of patients,there were 5 primary PCL(p PCL)and 1secondary PCL(s PCL);2 males and 4 females;aged 56-68 years,with a midian age of 67 years.2.The main clinical manifestations of PCL in this group included 2 cases of chest tightness,2 cases of bleeding,2 cases of bone pain,1 case of fever,1case of hepatomegaly,1 case of splenomegaly,1 case of lymphadenopathy,1cases of serosal effusion,and 1 case of jaundice.3.In the laboratory results,there were 5 cases of leukocytosis,6 cases of anemia,5 cases of thrombocytopenia,3 cases of creatinine increased,4 cases of LDH abnormality,3 cases of blood calcium increased,5 cases of rerythrocyte sedimentation increased and 6 casas of β2-MG abnormality.Serum immunofixation electrophoresis was performed in 5 patients.There were 1cases of Ig Gλ type,1 case of Ig Gκ type,1 case of Ig Dλ type,1 case of Ig Aκ type,1 case of λ light chain type;5 cases underwent flow cytometry,5cases expressed CD38,4 cases expressed CD138,1 cases expressed CD56,and all 5 cases did not express CD19 and CD20.4.According to the results of cytogenetics,there was only 1 case of s PCL with complicated chromosomal abnormalities.A total of 4 cases detected by FISH,1 case of s PCL was positive for Ig H rearrangement,TP53 and RB-1,Among p PCL,2 cases were positive for Ig H rearrangement,1 cases for RB-1,and 1 case for CKS1B/CDKN2 C.One p PCL patient underwent second-generation sequencing,which showed NRAS and DNMT3 a mutation.5.Among the 5 p PCL patients in this group,1 case did not receive chemotherapy,the remaining 4 patients received chemotherapy with BCD,TAD,VDT and VD regimens,respectively.1 case of s PCL was treated with melphalan+dexamethasone+lenalidomide after progression.6.In this group,1 case has a survival period of 13 months,2 case has 4months,1 cases have 3 months,1 case survival period was﹤1 months and 1case was lost to follow-up.Conclusions:Plasma cell leukemia is a kind of rare hematological malignancy with complex clinical manifestations,great heterogeneity of immunophenotype and cytgenetices.The disease progresses rapidly,has a poor prognosis,a short survival time,and some patients survive even less than 6 months.For high-risk MM with complicated chromosomal karyotype,its progession to s PCL should be vigilant.At present,bortezomib-based chemotherapies are the mainstream option for PCL.The patients suitable for transplantation should undergo HSCT as soon as possible.