Thalassemia, as a common genetic disease in Guangdong and other southern China, after years of publicity, control and treatment, its incidence and prevalence are being effectively controlled. But for the existing thalassemia patients, standardized blood transfusion and iron removal are still very important. Not to mention the danger of life, there is also the risk of paralysis caused by the formation of extramedullary hematopoietic tissue in the spinal canal to generate compressed nerves.
The Department of Spine and Orthopedics of the First Affiliated Hospital of Jinan University has recently treated two patients with intraspinal tumor. Both of them suffered from nerve compression symptoms such as numbness and weakness in their limbs, and the culprit of the accident turned out to be thalassemia.
Symptoms worsened after rare disease surgery 7 years ago
In another spinal canal Mr. Liu (pseudonym), a 43-year-old man who had grown hematopoietic tissue compressing nerves, came to the hospital 7 years ago because of “numbness and weakness in both lower limbs, strenuous walking, and weakness in squatting and legs”. Associate Professor Cha Dingsheng from the Department of Orthopedics of the First Affiliated Hospital of Jinan University visited the outpatient clinic and found a tumor in the thoracolumbar spinal canal through imaging examination. He was diagnosed as “thalassemia combined with intraspinal extramedullary hematopoiesis in the thoracic and lumbosacral segments”. Mr. Liu underwent decompression surgery for intraspinal extramedullary hematopoiesis in the thoracic canal, and Mr. Liu recovered well after the operation.
7 years later, Mr. Liu was admitted to the hospital again due to numbness of the soles of his feet. After completing the examination, the chief physician Cha Dingsheng found that Mr. Liu had no recurrence of extramedullary hematopoiesis in the thoracic spinal canal, but the lumbosacral spinal canal did not recur. The intramedullary hematopoiesis was significantly worse than 7 years ago, and the sacral 1 nerve roots were compressed on both sides. Since the numbness of the soles of the feet obviously affected Mr. Liu’s life and work, after communicating with Mr. Liu, Cha Dingsheng and his superior physician Wu Hao decided to implement minimally invasive unilateral dual-channel spinal endoscopic lumbosacral fenestration for Mr. Liu Decompression, extramedullary hematopoietic tissue removal. Mr. Liu’s operation went well, and the symptoms of numbness in the soles of the feet were significantly relieved after the operation, and he was discharged from the hospital 4 days after the operation.
Coincidentally, during the same period, a newly admitted patient from the Orthopedics Department of the First Affiliated Hospital of Jinan University had a highly similar condition. Hematopoietic tissue that should not have appeared in the thoracic spinal canal, which continuously expands and squeezes the patient’s nerves, causes weakness in both lower extremities, and difficulty urinating, which brings a lot of inconvenience to the patient’s life, and there is a risk of complete paralysis.
Problems of the hematopoietic system
How does it affect the nervous system?
Thalassemia, obviously a problem of the hematopoietic system, how can it be so high-risk that it affects the nerves in the spinal canal?
Wu Hao said that the liver and spleen are the main hematopoietic organs during the fetal period. After birth, the bone marrow becomes the main hematopoietic organ. When the human body suffers from a decrease in red blood cells, such as various hemolytic anemias, the extramedullary tissues such as the liver and spleen may partially restore the hematopoietic function to supplement the red blood cells that the human body lacks. Extramedullary hematopoiesis. It can be found in many different parts, such as liver, spleen, lymph nodes, kidney, paravertebral, retroperitoneum, pelvis, etc. In some cases, extramedullary hematopoiesis can form tumor-like lesions, and imaging studies show tumor-like infiltrative findings. It is extremely rare to form extramedullary hematopoietic tissues in the spinal canal (where the nerve lines are located). These hematopoietic tissues expand and the spinal nerves are squeezed, resulting in quadriplegia and other pre-paralysis symptoms of spinal cord compression.
Currently, the treatment methods for spinal cord compression include blood transfusion, radiation therapy, and surgery. Unilateral dual-channel spinal endoscopic surgery is a minimally invasive technique used in spine surgery, which combines spinal surgery technology and arthroscopy technology. Because of its wide surgical field, good viewing angle, and thorough decompression, it has been gradually carried out in China in recent years and is applied to the lumbar spine. Canal stenosis, lumbar disc herniation, lumbar spondylolisthesis treatment, especially in the treatment of spinal stenosis with technical innate advantages. It can completely remove the hypertrophic ligamentum flavum and hyperplastic osteophytes, and release the bilateral nerve roots while preserving the main stable structure of the spine. This technique is applied to the thoracic spine to open the spinal canal, remove a certain amount of extramedullary hematopoietic tissue, relieve the compression of the spinal cord and nerve roots, avoid massive intraoperative bleeding, aggravate anemia, and promote rapid recovery of thalassemia patients.
At present, hematopoietic stem cell transplantation is needed to cure thalassemia, and regular hemoglobin transfusion can alleviate the condition. Experts remind patients suffering from thalassemia to perform MRI examination of the spine when conditions permit, to exclude the phenomenon of extramedullary hematopoiesis in the spinal canal, and to choose an appropriate treatment plan according to the severity of the condition. For mild cases, regular blood transfusion therapy and radiotherapy can be selected. For severe spinal cord compression, surgical treatment such as unilateral dual-channel spinal endoscopic minimally invasive treatment can be considered.
Written by: Southern Metropolis reporter Wang Daobin correspondent Zhang Cancheng