Misdiagnosis analysis of POEMS syndrome and its early diagnosis / 第二军医大学学报

ABSTRACT

Objective:To study the causes of misdiagnosis of patients with POEMS syndrome and to discuss the clues for its early diagnosis.Methods:The clinical and laboratory data of 26 inpatients with POEMS syndrome,who were treated in Changhai hospital over the last decade,were retrospectively analyzed.Results:The misdiagnosis rate of our group was 100%. The misdiagnosis was made in(3.31?0.97)hospitals and in(3.31?0.93)clinical departments;the misdiagnosis period was (19.42?10.86)months and it had been misdiagnosed as 18 other diseases.The initial symptoms included polyneuropathy in 21 (80.8%)cases,edema of lower extremity in 22(84.6%)cases,and body weight loss in 8(30.8%)cases.The typical clinical symptoms included polyneuropathy in 26(100%)cases and organomegaly in 24(92.3%).Two cases had newly-identified uterine hypertrophy,one had adrenal gland hypertrophy,and one had gastric wall thickening mimicking advanced gastric cancer.Hypothyroidism,impotence,skin pigmentation and sclerosis occurred in 76.9%(20/26),60%(6/10),92.3%(24/ 26)and 65.4%(17/26)cases,respectively.Monoclonal plasma cell proliferation was documented in 18(81.8%);M proteins were positive in 14(63.6%)cases by serum immunofixation,and only 2(9.1%)cases also positive by serum protein electrophoresis.One patient was positive of M protein by urine immunofixation and one had abnormal infiltration of plasma cells in the gastric wall.Lymph node biopsy were performed in 8 patients and 3 were found to have Castleman disease;the other 5 cases had lymphocyte infiltration,with 3 complicated with plasma cell proliferation.Nerve biopsy in 4 cases all revealed axonal degeneration and one patient complicated with demyelination.Bone marrow biopsy in 5 cases revealed plasmacytosis in 2 cases and myeloma in one.Excessive radioactivity resorption was found in 10 of the 16 cases(62.5%)and abnormal plasma cells were detected in 2 cases by bone aspiration guided by radioisotope bone scan.Conclusion:Misdiagnosis of POEMS syndrome is very common.Polyneuropathy,edema of lower extremity and body weight loss are the common early symptoms of POEMS syndrome. Early diagnosis can be made by having an intimate knowledge of the progression of the disease and by detecting M protein through various approaches.