Clinical Analysis Of Patients With Multiple Myeloma With Light Chain Amyloidosis

Objective: To investigate the clinical features,treatment outcome and prognosis of patients with multiple myeloma(MM)with light chain amyloidosis.Methods: Fifty-three patients were hospitalized from Department of Hematology of Beijing Chaoyang Hospital(main campus and western campus)from July 2006 to July 2016.According to the diagnostic criteria 0f 2014 IMWG for MM and AL,they were newly diagnosed as these two diseases.The clinical data were analyzed retrospectively by SPSS 22 software.Results: For MM with light chain amyloidosis patients,the ratio of male to female is about 2.1:1(36/17).Their mean age is about 59.9years old.The distribution of MM type is λ light chain(28.3%),Ig G-λ(20.8%),k light chain((18.9%),Ig G-k(17.0%),Ig A-λ(9.43%),Ig D-λ(3,8%)and Ig A-k type(1.9%).The involved organs by amyloidosis is for kidney,heart,skin and soft tissue skin,soft tissue,digestive tract,liver,nervous system and lung is 38 case(71.70%),33 cases(62.26%),17 cases(32.1%),13 cases(24.5%),12 cases(22.6%),9 cases(17.0%),9 cases(17.0%)and 1 case,respectively.The median overall survival time(OS)is 12(1-102)months.OS for MM patients with ISS Ⅱ is 34(4-81)months,longer than that of patients with ISS Ⅲ 8(1-102)months(P <0.05).For amyloidosis patients,the OS was 38(10-81),8(1-102)and 1(1-2)months in the patients with stage I,II and III(Mayo stage of amyloidosis),respectively(P <0.05).OS was significantly shortened with the increase of staging.their OS is 37(8-102),12(1-75)and 5.5(1-38)months,these are significantly associated with 1,2,and more than 3 organs involved(P <0.05).With the increase in organ involvement,survival is shortened.The OS of the heart affected is 6(1-36)months,significantly lower than the other patients without heart involvement(40,8-102)months(P <0.05).The OS of patients with hypotension is 3(1-8)months,lower than that of patients without hypotension OS(1-36)months(P < 0.05).In addition,OS in high-risk genetic change patients is 6.5(2-10)months,less than the other patients(15,1-102)months(P <0.05).Bortezomib based regimens are more efficient than the non bortezomib based regimens(63.6% vs 28.6%)(P < 0.05),but no statistical significance in OS.Conclusion: Multiple myeloma with amyloidosis is rare in clinical practice.The most common involved organ is kidney,followed by heart.While MM patient suffered with amyloidosis,their OS was significantly shorter.With the ISS period and Mayo stage increase,the OS gradually shortened.Moreover,the more their organ involved,the worse the prognosis.The heart involvement has the worst prognosis,and the patients with hypotension is also worse.In addition,the prognosis of patients with high-risk genetic change is worse.Patients who received bortezomib based regimens were more effective but had not yet been able to benefit from survival.