A case of Long-term misdiagnosis POEMS syndrome / 中国综合临床

ABSTRACT

POEMS syndrome is a rare clinical disease related to plasma cell disease.It often involves multiple systems, but it has no specificity.It is relatively easy to diagnose the typical " quintuple syndrome" , including polyneuropathy, organomegaly, endocrine disorders, m-albuminemia and skin lesions.But in the process of clinical diagnosis and treatment, it is found that there are very few patients with typical manifestations, so it is easy to lead to missed diagnosis or misdiagnosis.In this case, numbness and weakness of both legs were found at first, then pain of foot and back, swelling of whole body, pigmentation of skin, impotence and other symptoms gradually appeared.He has been successively hospitalized in spine surgery and pain department of Baiyin first people′s hospital.Considering Guillain Barre syndrome, myofascitis and lumbar disc herniation, the effect of symptomatic treatment is not good.After that, he gradually developed chest tightness, shortness of breath and pleural effusion.In addition, he was hospitalized in the respiratory department.He considered chronic obstructive pulmonary disease and pulmonary hypertension.During the examination, he found superficial lymphadenopathy.He underwent neck lymph node biopsy and considered lymphadenoplasmacytoma.Then he went to the hematology department to be hospitalized, and further improved the bone marrow aspiration smear, immunofixation electrophoresis, blood light chain protein determination, myeloid differentiation factor 88 (MyD88), vascular endothelial growth factor (VEGF) and other tests.Finally, he was diagnosed as poems syndrome.After diagnosis, he received systemic chemotherapy, and the condition was significantly relieved.