Clinical Analysis Of Multiple Myelomawith Extramedullary Disease And Exploration Of Its Mechanism

Objective: To retrospectively analyze the clinical data of 96 patients with extramedullary disease(EMD)and 80 patients without EMD of multiple myeloma in our hospital.The clinical features,efficacy,and prognosis of patients with multiple myeloma associated with strict extramedullary disease(sEMD),bone-raleted extramedullary disease(b EMD),and those without EMD were analyzed.Analyze the expression of protein and gene in the intramedullary and extramedullary lesions of some patients.Methods: Clinical data including 96 cases of multiple myeloma diagnosed with EMD and 80 cases of multiple myeloma without EMD were collected from January 2008 to January 2018 in Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology.Immunohistochemical staining was used to detect the expression of Ki-56,P53 and CD56 in six patients with intramedullary and extramedullary lesions.Fluorescence in situ hybridization was used to detect the expression of 1q21,13q14,17p13,t(11;14),t(4;14)and t(14;16).Results: Patients with sEMD had lower hemoglobin levels and higher lactate dehydrogenase and β2 microglobulin levels than those with b EMD and slao had higher lactate dehydrogenation than patients with multiple myeloma without EMD.Patients with b EMD had higher hemoglobin,higher albumin,lower creatinine value,lower β2 microglobulin,lower plasma cell ratio and lower ISS stage than patients without EMD.The median survival time(OS)for the sEMD group,the b EMD group,and the nonEMD group were 28 months,42 months and 44 months;the median progression-free survival(PFS)was: 15 months,26 Months and 29 months.OS and PFS of sEMD group were significantly lower than b EMD group and without EMD group.The expression rates of Ki-67,P53 and CD56 in bone marrow were 33%,8% and 75%.The expression rates of Ki-67,P53 and CD56 in extramedullary lesions were 80%,67% and 25%.Four of the five patients had inconsistent results of FISH between bone marrow and extramedullary lesions.13q14 deletion was found in the extramedullary lesion of four patient,while none of the four patients had 13q14 deletion in the bone marrow.Conclusion: Compared with b EMD,sEMD is more difficult to diagnose.It is necessary to take active examination to confirm the diagnosis.sEMD were one of the indicators of poor prognosis of multiple myeloma.We found that Ki-67 and P53 were highly expressed in extramedullary lesions,CD56 expression was down-regulate and extramedullary lesions had more gene abnormalities than in intramedullary lesions.It is speculated that the occurrence of extramedullary lesions may be closely related to the down-regulation of adhesion molecules,the high proliferation of malignant plasma cells and the genetic abnormalities,such as 13q14 deletion.