How is primary amyloidosis treated? Because the root cause of primary amyloidosis is the malignancy of plasma cells in the blood, the fundamental treatment is to remove the malignant plasma cells from the body. At present, there are three better ways to remove malignant plasma cells, the first is autologous hematopoietic stem cell transplantation, the second is general chemotherapy, and the third is new drug chemotherapy. Let’s start with autologous stem cell transplantation. Simply put, the patient’s own blood stem cells are isolated by machine and frozen in vitro; then the patient is given a mega-dose of chemotherapy, mainly using Marfalan; after the mega-dose of chemotherapy, the patient’s own frozen blood stem cells are transfused back into the body at an interval of two days. After about two to three weeks, the transfused stem cells will rebuild a hematopoietic system in the patient’s body, allowing his blood image to gradually return to normal, after which the patient can be discharged home for regular follow-up. Autologous stem cell transplantation for primary amyloidosis is one of the most reliable treatment methods with the highest number of cases in China and abroad. There is also a general chemotherapy, which is usually treated with normal doses of Marfalan plus doses of dexamethasone. There is also a new drug, bortezomib, which is also used to treat primary amyloidosis. The current international and national literature shows that all three therapies are very effective. So how do we go about choosing? After the disease is diagnosed, a thorough evaluation should be done to determine whether the patient is suitable for autologous stem cell transplantation. Because autologous stem cell transplantation has certain toxic side effects that not all patients can tolerate, we have a strict set of entry criteria for patients who need stem cell transplantation. If, after a thorough evaluation, the patient is not suitable for HSCT, chemotherapy or the new drug bortezomib can be chosen as treatment. Will the combination of autologous peripheral stem cell transplantation + chemotherapy + bortezomib together improve the cure rate? Yes, it will. The process of autologous stem cell transplantation actually involves high doses of medication. What are the side effects or sequelae of the treatment process? The most significant side effect of autologous stem cell transplantation is the toxic side effect of high dose chemotherapy drugs. The main side effects of high doses of Marfalan are liver and kidney toxicity and bone marrow suppression. First of all, some patients may worry that the liver and kidney damage will be further aggravated by the already damaged liver and kidney, plus the effect of high-dose drugs. In fact, this is only a temporary aggravation, once the treatment takes effect, then the liver and kidney function will also improve. Secondly, the patient’s bone marrow will be suppressed after the high dose of drugs, and his white blood cells and platelets will be significantly reduced. After the autologous stem cell transfusion, the patient will experience two to three weeks of leukopenia or deficiency and thrombocytopenia, which exposes the patient to various risks of infection and bleeding. Can the patient recover after kidney transplantation? Is there a risk of recurrence? It is a very complicated issue to decide whether to do a kidney transplant in primary amyloidosis patients with renal failure. This is because, although the kidney function of the patient will improve significantly in the short term after successful kidney transplantation and the patient’s physical condition will improve accordingly, the transplanted kidney will be gradually destroyed by amyloid if the primary disease is not controlled. And doing hematopoietic stem cell transplantation treatment in the case of renal insufficiency is very risky, which makes people torn. Therefore, the most fundamental treatment is still aimed at the removal of malignant plasma cells, in addition, whether to do a kidney transplant and whether to do a kidney transplant should listen to the urologist’s opinion. What about patients with heart failure? Patients with primary amyloidosis who develop heart failure are more difficult to treat. Heart failure in this type of patient is mainly diastolic failure, so the treatment is based on calcium antagonists and beta-blockers, appropriate diuretics, and generally no cardiac drugs like digitalis. In fact, we are more concerned about cardiac problems in patients with primary amyloidosis than arrhythmias, which have been reported in the literature to cause sudden death due to sudden onset of malignant arrhythmias.