OBJECTIVETo evaluate the usage of Mayo staging system in Chinese patients with primary light chain (LC) amyloidosis.

METHODClinical data, treatment and outcome of 162 primary LC amyloidosis patients with Mayo Clinic staging in Peking Union Medical College Hospital from January 2009 to June 2015 were retrospectively analyzed.

RESULTSThe median age of 162 patients with Mayo Clinic 2004 stage was 57 (20-81) y, of them 62.3% were male. The number of patients with stage I to III were 44 (27.2%), 69 (42.6%), and 49 (30.2%), respectively. The median overall survival was not reached, 23 months and 12 months in patients with Mayo Clinic 2004 stage I, II, and III, respectively (P<0.001). Among 128 patients with Mayo Clinic 2012 stage, 48 patients (37.5%), 32 patients (25.0%), 32 patients (25.0%) and 16 patients (12.5%) were staged as Mayo Clinic 2012 stage 1 to 4, and the median OS was not reached, not reached, 13 months and 3 months, respectively (P<0.001).

CONCLUSIONMayo Clinic staging systems had important prognostic value in patients with primary LC amyloidosis.

Adult ; Aged ; Aged, 80 and over ; Amyloidosis ; diagnosis ; Female ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Young Adult

Objective: To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China. Method: Clinical data, treatment and outcome of 205 pAL patients in Peking Union Medical College Hospital from January 2009 to February 2016 were retrospectively analyzed. A 'very high risk’ group includes patients with Mayo 2004 stage Ⅲb and Mayo 2012 stage 4. Results: Of 205 patients, 34 (16.6%) were defined as very high risk pAL patients. The median age at diagnosis was 57 (20-84) years, and 22 patients (64.7%) were male. All 34 patients were diagnosed with cardiac involvement, multi-organ involvement was observed in 15 patients (44.1%) , and 27 (81.8%) had New York Heart Association Class Ⅲ or Ⅳ. Median values of serum cTnI, NT-proBNP, and free light chains difference were 0.25 μg/L, 11 733 ng/L, and 403 mg/L, respectively. Eight (24.2%) had more than 10% plasma cell on the bone marrow aspirate. Sixteen (47.1%) patients received bortezomib based chemotherapy and overall hematologic response rate was 58.3%. Median overall survival (OS) was 4 months. The estimated OS at 3, 6, 12, and 24 months was 51.3%, 44.0%, 35.2%, and 29.6%, respectively. Fourteen (41.2%) patients died within 3 months after the diagnosis. The estimated 1-year survival rate for the patients who got hematologic response, without hematologic response, and palliative treatment was 90.9%, 11.1%, and 0, respectively (P<0.001) . Conclusion Patients with very high risk pAL had very poor prognosis and the early death rate remained high. Those patients who obtained hematologic remission would have significantly better outcomes.

Objective To study the use of laparoscopy in the diagnosis and treatment of obstructive infantile cholestasis.Methods The clinical data of 106 patients with obstructive infantile cholestasis from January 2012 to June 2017 were studied retrospectively.After two weeks of conservative treatments which failed to decrease the bilirubin levels significantly,these patients were subjected to laparoscopic diagnosis and treatment.Results A correct diagnosis was established in all these 106 patients by laparoscopic biliary tract exploration and cholangiography.Eighty-eight patients were diagnosed to have biliary atresia (83.0％),16 patients inspissated bile syndrome (15.1％) and 2 patients biliary hypoplasia (1.9％).Thirty-eight of the 88 biliary atresia patients gave up operative treatment after laparoscopic biliary tract exploration and cholangiography.The remaining 50 biliary atresia patients were treated with open Kasai portoenterostomy.The prognosis of the biliary atresia patients were different from the non-biliary atresia patients.On follow-up for 4 months to 5 years,all the 18 non-biliary atresia patients were in good condition and there was no recurrence of jaundice after laparoscopic cholecystostomy and biliary tract irrigation.Conclusions The laparoscopic minimally invasive technique helped to establish diagnosis and treatment in patients with obstructive infantile cholestasis.For patients with biliary atresia,this procedure gave a definitive diagnosis and offered an opportunity for surgery.For patients with inspissated bile syndrome and biliary hypoplasia patients,laparoscopic cholecystostomy and biliary tract irrigation established the correct diagnosis and reduced liver damage resulted by cholestasis.