| Objective To analyze the clinical characteristics of POEMS syndrome,to explore the main clinical manifestations of POEMS syndrome and the influential factors of related examination results,to improve doctors’ s awareness of POEMS syndrome,and thus reduce the rate of misdiagnosis and misdiagnosis of the disease.Method Retrospective analysis of the medical records of 14 patients diagnosed with POEMS syndrome admitted to the ward of Tianjin Medical University General Hospital from January 2009 to September 2019.The main clinical manifestations,related examinations,treatment options and outcomes of POEMS syndrome were analyzed.Results The 14 patients with POEMS syndrome were classified into 9 male patents(64.3%)and 5 female patients(35.7%).The age distribution was from 44.0 years to 65.0 years,with a mean value of 58.6 years.The time from the onset of symptoms to the first visit to hospital was 1-36 months,with an average of 11 months.Of the 14 patients with POEMS syndrome,2 had skin pigmentation as the first symptom,5 had limb numbness as the first symptom,6 had abdominal distension,lower extremity edema,and chest tightness as first symptoms which are of an increase in extravascular volume load.Nine patients with numbness and weakness(64.3%)of the lower extremities,which were the most common symptoms of peripheral neuropathy in POEMS syndrome;organ enlargement was mainly manifested by the enlargement of the liver,spleen,and lymph nodes;low testosterone levels in 4 male patients(44.4%),and low testosterone levels mean lower than the lower limit of normal value,10 cases(71.4%)had hypothyroidism,and 8 cases(57.1%)had adrenal insufficiency.Among skin lesions,skin pigmentation in this group of cases is the most common,there were 10 cases in all(71.4%),2 cases(14.3%)had abnormalities of sweat secretion(mainly hyperhidrosis),4 cases had thickened hairs(28.6%),and 11 cases had extremities and facial edema(78.6%).5 cases(35.7%)had pleural effusion,7 cases(50.0%)had ascites,4 cases(28.6%)had pericardial effusion,6 cases(42.9%)had papilledema;M protein classification: 13 of 14 patients routine immunofixation electrophoresis were performed.The light chains were all Lamda positive,6 were heavy chain Ig A positive(42.9%),6 were Ig G positive(42.9%),and no heavy chain type was detected in 1;of 11(78.5%).)patients,bone marrow smear was performed.Nine patients underwent biopsy at the same time.Bone marrow plasma cells were clustered in one case.There were 3 cases who had a fixed numerical value,respectively 1.5%,1.5%,4%.Granulocyte,erythroid,megakaryocyte hyperplasia of four cases,two cases of bone marrow hypoplasia.Regarding treatment options,5 patients received lenalidomide combined with glucocorticoids(35.7%),2 patients received bortezomib combined with glucocorticoids(14.3%),and 2 patients received glucocorticoid therapy alone(14.3%),Short-term chest tightness,belching,edema,etc.were alleviated,but the long-term effect was not ideal and recurrence occurred.One patient(7.14%)received peripheral blood autologous hematopoietic stem cell transplantation(ASCT)after chemotherapy.A 60-year-old male patient developed severe haematological depression and severe anemia after using lenalidomide.He was automatically discharged from the hospital after blood transfusion treatment and had a poor prognosis. |
