Abstract: Ｏｂｊｅｃｔｉｖｅ：To analyze the clinical features and prognosis of systemic light chain amyloidosis associated with Immunoglobulin M(IgMAL).
Ｍｅｔｈｏｄｏｌｏｇｙ：We retrospectively analyzed the general situation,clinical manifestations,laboratory examination features, treatment responses and prognosis of 17 patients with IgMAL.
Ｒｅｓｕｌｔｓ：The median age was 61 (49~75) years,17 patients (100％) had renal involvement and 8 patients (47％) had cardiac involvement.Serum immunofixation electrophoresis showed that 13 cases were λIgM,4 cases were κIgM,and 15 cases (882％) had elevated serum IgM concentration.Comparied with IgGAL patients,IgMAL had more lymph node involvement (176％ vs 3％,P=0018),lower percentage of bone marrow plasma cells (10％ vs 32％,P<0001), lower abnormal rate of plasma cell interphase florescence in situ hybridization(143％ vs 653％,P=0031),lower urinary retinol binding protein (03 mg／L vs 06 mg／L,P=0026), and higher serum IgM (797 g／L vs 079 g／L,P<0001).After chemotherapy and／or high dose melphalan and autologous stem cell transplantation, the overall hematologic response rate was 667％ and the deep hematologic response rate was 333％, but the complete response rate was low (83％). The 2year survival rate of patients was 76％. Patients with severe heart involvement had poor prognosis.
Ｃｏｎｃｌｕｓｉｏｎ：λ light chain abnormal was more common in IgMAL, with higher lymph nodes involvemen, lower load of bone marrow plasma cells and genetic changes when compared with IgGAL.Antiplasma cell therapy is effective,but complete hematologic response rate was low.The severity of cardiac involvement is a major prognostic factor.