1. Case data The mean age of the patients was 55(39-72) years, 7 males and 4 females; 10 of them were clearly amyloid: κ-type 1 case, λ-type 9 cases. 5 cases were positive for immunofixation electrophoresis: IgG κ-type 1 case, IgG
λ-type 3 cases, IgA λ-type 1 case. The organs involved were judged according to the literature criteria: kidney 100%, intestine 81%, liver 64%, heart 54%; median disease diagnosis time was 5 months; ECOG score was 3 in 5 cases, 2 in 4 cases and 1 in 2 cases. Zhai Yongping, Department of Hematology, General Hospital of Nanjing Military Region Seven cases were initially treated and four cases were refractory – three of them received autologous hematopoietic stem cell transplantation and were ineffective, and one patient progressed after 8 courses of Marfalan combined with high-dose dexamethasone, 2 courses of chemotherapy with VAD regimen, and improvement with thalidomide. 2. Treatment regimen Bortezomib combined with dexamethasone was used in a two-week dosing regimen: bortezomib 1.3 mg/m2 was administered rapidly intravenously over 3-5 s on days 1, 4, 8 and 11. Dexamethasone 20-40 mg in 100 ml saline was given intravenously before each intravenous bortezomib injection. If platelets are less than 30×109/L on the day of administration or if there is infection, the dose is temporarily postponed and continued after platelets rebound or infection is controlled. If intolerable or dangerous complications occurred, the drug was discontinued. RESULTS 1. Efficacy observation Eight of 11 patients received bortezomib combined with dexamethasone for 1-6 courses of treatment, with a median of 3 courses and a median follow-up of 6 (3-15) months. 7 patients obtained different degrees of efficacy, with an overall efficiency of 87%, an organ response rate of 75%, and improvement in organ function in the order of kidney, liver, and intestine. The median onset of effect was 2 months. 2, adverse reactions The most common adverse reaction was gastrointestinal symptoms, among 11 patients, 10 cases showed diarrhea and loss of appetite, of which 6 cases showed severe diarrhea (all appeared in more than 1 course of treatment), all reached NCI
CTCAE classification grade 3, which improved with symptomatic treatment and delayed or discontinued medication. No constipation occurred. Thrombocytopenia occurred in 8 patients, and only 1 patient reached CTCAE grade 4. Platelets were 140×109/L before treatment and 9×109/L on day 3 at the end of treatment, which was supported by platelet transfusion and recovered to 20×109/L and pre-treatment levels on days 6 and 10 after the end of treatment, respectively. The rest of the patients with thrombocytopenia mostly returned to baseline levels 3-6 days after drug discontinuation, with CTCAE grade 1 to 2.
No cases with significantly fewer white blood cells and significantly decreased hemoglobin were found. Peripheral neuropathy such as numbness at the ends of the extremities was observed in 6 cases, including 1 case in which the numbness reached the hip and affected walking after 3 courses, with CTCAE grade 3 and grade 2 in the remaining cases. There were 5 cases of herpes zoster associated with drug administration. The symptoms of weakness and weakness were gradually relieved after stopping the drug in 5 cases. 2 cases of postural hypotension and syncope occurred, and 1 case of 35% left heart ejection fraction without obvious signs and symptoms of heart failure occurred after the 3rd dose of the 5th course of treatment, and the condition was controlled by resuscitation. In another patient with hepatic, renal and intestinal involvement, total bilirubin increased from 36.5umol/L to 115.8umol/L in the second course, with simultaneous increase in direct and indirect bilirubin, and transaminases were basically normal, which gradually improved after stopping bortezomib.