57 -year -old Ling Shu, who has repeatedly swollen and pain in both hands in the past one year, has not paid attention to it. In the past three monthsThe skin of both fingers was cracked, and multiple hospitals have been traveled one after another.The patients condition worsened every day and was very anxious. What strange disease, wont get it , right?
After the patient stays in Hunan Provincial Hospital of Traditional Chinese Medicine , Director Xiao Feng of the Cancer (Hei Oncology) When carefully looking at the patients medical history data, it was found that the patients blood in the blood was abnormally elevated, the proportion of albumin and globulin was inverted, and the urine protein was positive, and it was highly suspected of abnormal monoclonal globulin.
Immediately improve the serum protein electrophoresis, immune fixed electrophoresis, bone marrow puncture and biopsy, which is confirmed that the patient is: IGG λ type bone marrow tumor, cardioprocnocioplasia: ventricular wall, two -pointed valve, three -pointed valve, three -pointed pointed, three -pointed pointed, three -pointed pointed, three -pointed pointed, three -pointed -pointed pointed, three -pointed pointed, three -pointed -pointed pointed, three -pointed -pointed pointed, three -pointed -pointed -pointed pointed, three -pointed -pointed pointed, and three -pointed valve.It is obviously thickened with dust, considering the possibility of cardiopathy, and further improve the skin biopsy: (abdominal fat) starch -like substance deposits, Congo red staining (+).
At this point, the truth is white. Uncle Ling is suffering from Rare hematopic disease: systemic light chain starch -like degeneration (multi -multiple osteoma secondary) .After several months of regular chemotherapy+targeted therapy, Uncle Lings chest tightness was significantly improved, his hands were swollen and reduced, and the skin of the skin gradually faded.
What are the clinical manifestations of systemic light chain starch-like degeneration?
Systemic light chain starch -like degeneration is not related to eating too much food starch, because the single cloning immunoglobulin light chain error folding is formed to form starchSample protein, deposited in the whole body tissue and organs, causing diseases that cause tissue structure, organs, dysfunction, and progressive progress.
Based on whether the blood tumor is combined, it can be divided into primary and secondary.Secondary light chain type degeneration is secondary to other plasma cell/B cell diseases, such as multiple osteoma and lymphoma.
It is a rare blood system. According to relevant statistics, the incidence of the Chinese population is about 30/1 million.Various clinical manifestations can affect multiple organs.Kidney and heart are the most commonly involved organs. Other accumulated organs include liver, autonomy or peripheral nerves, digestive tract, and soft skin tissue.
What are the treatment methods of systemic light chain starch -like degeneration?
Once the patient is diagnosed, the patient should start treatment as soon as possible in accordance with the pre -pre -stages, the function of the involved organ, the physical condition, and the obtained drugs.
The treatment goal is to reduce the level of light chain of the single cloning immunoglobulin in the body, prevent starch -like protein from further depositing in important organs, and reducing or reversing organ function caused by starch -like protein depositionobstacle.
With the rapid progress of medicine in recent years, light -chain starch -like drugs have continued to emerge, and targeting, chemotherapy, immunization and other treatment methods can be used , at the same time can cooperate with Chinese medicine auxiliary to reduce treatment related adverse reactions.For eligible patients, autologous hematopoietic stem cell transplantation is a good consolidation treatment method.
What are the precautions for patients with systemic light chain starch -like transformer?
The care of starch -like degeneration involves multiple aspects, depending on the specific types of starchy degeneration and the affected organ system.
Skin care:
Skin care products, choose mild cleaning and moisturizing products.
2. Keep the skin dry and avoid hot water washing and scratching to prevent skin damage and infection.
3. Wear loose and soft clothing to reduce skin friction.
Diet adjustment:
1. Adjust the dietary structure according to the type and condition of starch -like degeneration.For example, patients with starchy degeneration in kidney need to limit protein intake during renal failure.
2. Keeping low salt and low -fat diet can help control edema and other related symptoms.
Observation and support:
1. Regularly monitor the life signs and condition changes of patients, especially for changesPatients with starchy degeneration need to closely monitor heart rate, blood pressure, and heart function. Usually getting up should be slow to avoid orthogical hypotension.
2. Provide psychological support to help patients and family members cope with the psychological pressure caused by the disease.
Prevent complications:
Strengthen cleaning and care to avoid infection.
2. For patients with possible thrombosis, such as patients with starchy degeneration, should be encouraged to raise lower limbs, carry out light sports activities such as Tai Chi and BabuStupid, regular monitoring coagulation function.
Hunan Medical Chat Special Author: Hunan Provincial Traditional Chinese Medicine Hospital Tumor Second Department of Tumor Hu Yarong Follow@Hunan Medical Chat, get more health science information!
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