Autologous hematopoietic stem cell transplantation for POEMS syndrome

POEMS syndrome, as a clinically rare clonal plasma cell disease, has disadvantages in treatment such as difficulty, high disability rate, short patient survival (2-7 years) and poor prognosis. Peking Union Medical College Hospital has been the first to use autologous peripheral blood hematopoietic stem cell transplantation to treat POEMS syndrome in China since 2005, and has completed 22 autologous transplants so far, achieving remarkable efficacy. The number of transplantation cases reported abroad is only 38 cases. This achievement was awarded the second prize of medical achievement in 2010 by Peking Union Medical College Hospital. POEMS syndrome is a rare disease caused by plasma cell abnormalities in peripheral nerve, endocrine, blood, gastrointestinal, skin, bone, kidney, plasma membrane, etc. It was named POEMS syndrome in 1980, based on its polyneuopathy (P), organomegaly (O), endocrinopathy (O), and endocrinopathy (O). Endocrinopathy (E), M-protein (M) and skin changes (S). Currently, glucocorticoids, cytotoxic drugs and local radiation therapy are mainly used, but the prognosis is poor, with a disability and death rate of almost 100%. Multidisciplinary collaboration and intensive clinical research have proven that autologous peripheral blood HSCT is an effective and safe treatment for POEMS syndrome, which can maximize the improvement of symptoms and provide patients with a high quality of life. In 2006, Ms. Xu, who is in her 30s, was diagnosed with POEMS syndrome when she came to Concordia Hospital with unexplained foot drop and darkening skin. After analysis, Concord Hospital performed an autologous peripheral blood stem cell transplant for Ms. Xu. Due to early detection and timely treatment, Ms. Xu’s neurological function was mostly restored after the surgery. Six months later, seeing the hope of surgery, Ms. Xu had a second autologous transplant, and her nervous system basically recovered, with no recurrence to date. ? Professor Zhou Daobin said that the core of autologous transplantation is high-dose chemotherapy, and the transfused autologous hematopoietic stem cells are mainly used to restore hematopoietic function. The basic procedure is to collect enough HSCs from peripheral blood and freeze them in a low-temperature refrigerator. After the patient is treated with mega-dose chemotherapy at an appropriate time, the HSCs are re-infused back into the patient. During the autologous transplantation process, the mega-dose chemotherapy removes the abnormal plasma cells that cause POEMS syndrome and also destroys the patient’s own immune and hematopoietic systems, requiring the patient to enter a sterile laminar flow room for protective isolation. Within 2 to 3 weeks after the return transfusion, the hematopoietic and immune functions are largely restored and the patient can leave the laminar flow ward. If chemotherapy breaks the old life, transplantation creates a new world for the patient. The successful implementation of this work fully reflects the advantages of our strong comprehensive strength and smooth multidisciplinary collaboration, and opens up new ways for the diagnosis and treatment of difficult and rare diseases.