Primary myelofibrosis (PMF) is a chronic clonal myeloid disease characterized by anemia, splenomegaly, immature granulocytes, juvenile erythrocytes, teardrop erythrocytes and CD344 cells in the peripheral blood, myelofibrosis and osteosclerosis.The incidence of PMF is about 0.5/100,000 and occurs mainly in the elderly population.The WHO classifies PMF as a BCR/ABL negative myeloid PMF is still an incurable disease with a median survival time of 4-7 years, and the prognosis varies widely among patients. Our country is characterized by younger patients, a high percentage of anemia occurring at the initial diagnosis, and more severe anemia. Patients with hemoglobin less than 80 g/L have a significantly shorter survival time. There is a lack of ideal treatment for this disease. I. Conventional drug treatment 1. Improvement of anemia: Treatment is started when hemoglobin is <100g/L. Conventional drugs include glucocorticoids, androgens, erythropoietin and immunomodulators, and appropriate drugs are selected according to the patient's age and tolerance. Patients with initial treatment can be treated with a combination of glucocorticoids and androgens for at least 3 months. If the treatment is effective, androgens are continued, but the amount of corticosteroids needs to be gradually reduced. Erythropoietin is indicated for patients with EPO <100 U/L. It is less effective in transfusion-dependent patients and may aggravate splenomegaly when used in patients with moderate or greater splenomegaly. Severe anemia may be treated with red blood cell transfusions. The literature reports that for transfusion-dependent patients, multiple transfusions need to be treated with de-ironing, which may reduce the number of transfusions. The main effects of immunomodulators are anti-angiogenesis, down-regulation of TNF-α and IL-6, up-regulation of IL-2 and TNF-α and enhancement of T cell and NK cell proliferation and activity. One of the more common drugs used to improve anemia and thrombocytopenia in PMF patients is thalidomide, starting with a small dose (50-100 mg/d) and slowly increasing the dosage. However, high doses of thalidomide (100-600 mg/d) are associated with adverse effects such as constipation, drowsiness, headache, and neuritis, and most patients have difficulty tolerating them and withdraw from treatment. To avoid these adverse effects, the investigators used low-dose thalidomide (50 mg/d) and combined prednisone treatment, which was able to reduce the adverse effects while shortening the duration of adverse effects. Lenalidomide, a derivative of thalidomide, has been replacing thalidomide in recent years. It not only has an enhanced inhibitory effect on TNF-α, but also has significantly reduced adverse effects and is effective in treating PMF with 5q-cytogenetic abnormalities. Another derivative of thalidomide, pomalidomide, is a 3rd generation immunomodulator with major adverse effects including neutropenia and thrombosis. The main adverse effects include neutropenia and thrombosis, with significantly lower neurotoxicity than thalidomide and significantly lower myelosuppressive effects than lenalidomide. A recent phase II clinical trial conducted by Begna et al. showed that a low dose (0.5 mg/d) was significantly more effective in JAK2-V617F-positive patients than in negative patients. 2. Reduction of splenomegaly (1) Chemotherapy: Drugs recommended by the European Leukemia Network include cladribine (5mg/m2 daily, 2h infusion for 5d, 1 course per month, repeated for 4-6 courses), marfalan (2.5mg, 3 times per week), and leucovorin (2-4mg/d). It should not be overlooked that hydroxyurea is naturally the first choice of chemotherapy with a controlled dosage of 20-30mg/kg and a spleen reduction efficiency of about 40%. However, adverse effects such as anemia or abnormal red blood cell morphology, leukopenia and thrombocytopenia can occur with hydroxyurea. (2) Radiation therapy: Radiation therapy can be chosen if the patient has one of the following symptoms: severe pain in the splenic area, significant enlargement of the spleen, contraindication to splenectomy, symptoms of spinal cord compression, and symptoms of extramedullary fibrous hematopoietic tumors. The advantage of radiation therapy is that it has a significant spleen reduction effect. The disadvantage is that the duration of action is not long-lasting, about 3-6 months. (3) Splenectomy: Since the spleen is an extramedullary hematopoietic organ, removal of the spleen may cause rapid enlargement of the liver and dramatic increase of platelets, so this method has certain limited population. This method is suitable for: ① if the giant spleen shows obvious compression symptoms followed by persistent splenic infarction, thus causing persistent pain. (ii) Patients with a drastic decrease in platelets or with hemolytic symptoms due to hypersplenism, which can be used when long-term drug therapy has failed. In contrast, for those with high platelets and who are prone to intravenous thrombosis after surgery, such patients are generally considered as contraindications to splenectomy. Splenomegaly is a poor prognostic factor for patients proposed for hematopoietic stem cell transplantation, so some scholars suggest that patients with significantly enlarged spleens should be splenectomized before transplantation. Allogeneic hematopoietic stem cell transplantation Currently, the only possible cure for PMF is allogeneic hematopoietic stem cell transplantation (allo-HSCT), but it is risky, has a high rate of postoperative complications (chronic graft-versus-host disease, infection, etc.) and death, and there is no conclusive evidence that allo-HSCT is associated with prolonged survival. The results of a large retrospective analysis of 289 PMF patients suggest a 27%-43% mortality rate of allo-HSCT between non-blood donors over 1 year. Cervantes et al. and Dingle et al. proposed the principle of selecting treatment options based on patient age and prognostic risk, and both concluded that allo-HSCT should be performed in high-risk patients. allo-HSCT remains the first-line treatment option for patients in the intermediate-risk-2 group because survival is less than 3 years. Patients in the low-risk group with long survival can be temporarily treated with symptomatic support. According to studies, an important factor for cure is age, and the 5-year survival rate for clear-medullary allo-HSCT is only 14% for patients aged >45 years, compared to 62% for patients under 45 years. However, the majority of PMF patients are over 50-60 years of age and have a high risk of death from clear-medullary allo-HSCT. The finding of comparable efficacy of clear- and non-clear-medullary allo-HSCT has led to the introduction of various reduced intensity preconditioning (RIC) regimens for partially clear-medullary to non-clear-medullary, which have been successfully used in PMF patients who cannot receive clear-medullary SCT due to advanced age or comorbidities. In a retrospective RIC study, 21 patients with a mean age of 54 years, intermediate/high risk, pretreated with different RIC regimens and receiving allo-HSCT from a kindred donor, had a non-relapse mortality (NRM) rate of 10% at year 1 and an overall survival rate of 85% at 2.5 years. In a recent prospective study of RIC, 103 patients (mean age 55 years) pretreated with low-dose leucovorin/fludarabine had a non-relapse mortality rate of 16% at 1 year, 32% experienced chronic graft-versus-host disease, and the overall survival rate was 70% at 3 years, with an overall relapse rate of 22% at 3 years. It is evident that RIC may reduce the rate of transplant-related morbidity and mortality and improve the prognosis of patients with allo-HSCT. In recent years, data from the International Center for Blood and Marrow Transplantation Research show a gradual increase in the number of allo-HSCT performed, which is considered to be related to the reduction of the intensity of pretreatment regimens and the opportunity to perform allo-HSCT in elderly PMF patients and patients with comorbid high-risk diseases. In addition, the use of JAK2 inhibitors improves the quality of survival in patients with MF and may also increase the number of patients who can undergo allo-HSCT. The timing of transplantation is equally important for outcome. In clinical practice, having patients undergo stem cell transplantation is a last resort treatment. However, if the patient has adverse cytogenetic (especially chromosome 17) abnormalities and/or clinical manifestations of disease progression (e.g., primocytosis, severe thrombocytopenia) or turns to acute leukemia with a prognosis of survival of < 1< span="">year, the urgent need for stem cell transplantation cannot be delayed. Some studies have shown that the success rate of allo-HSCT during the fibrosis stage is 50-80%, but the transplantation success rate drops to 25-40% after the occurrence of leukemic transformation. Therefore, patients with PMF should be evaluated for risk as early as possible, and early transplantation of stem cells with appropriate protocols in patients with poor prognostic scores and advanced age is crucial to improve the degree of fibrosis, reduce transplant-related morbidity and mortality, and prolong survival time. The current pharmacological treatment is basically palliative and cannot change the progression of PMF, much less achieve a complete cure for PMF, so researchers have tried to use a variety of new drugs, using a clinical trial approach to test the feasibility of drugs, of which JAK2 inhibitors are a relatively successful representative. The commonly used JAK2 inhibitors are divided into 2 types: specific (class I) and non-specific (class II). Some have been started in patients with intermediate to high-risk PMF. Chinese medicine treatment This disease is a refractory hematologic disease, the etiology of which is not clear, and Chinese medicine treatment is rare. Cai Yinyan et al. reported that the treatment of 12 cases of PMF with the method of clearing heat and detoxifying, activating blood circulation and removing blood stasis, and softening hardness and dispersion resulted in one case of improvement, eight cases of progress, and two cases of ineffectiveness, with two cases of disappearance of clinical symptoms, seven cases of significant improvement of clinical symptoms, and three cases of insignificant improvement after treatment, suggesting that the method of activating blood circulation and removing blood stasis has certain efficacy in treating PMF. In 2014, Xu Junqing et al. reported the application of low-dose thalidomide and prednisone as an effective treatment regimen for anemia in PMF patients, and the combined application of danazol significantly prolonged the duration of the therapeutic effect. Chen Shengmei et al. reported that interferon α-2b combined with thalidomide for high blood counts and prednisone combined with thalidomide for low counts had comparable therapeutic effects and could be an effective treatment for primary myelofibrosis.