How does hydroxyurea treat polycythemia vera?

Polycythemia vera is a myeloid blood disease with an unexplained abnormal proliferation of red blood cells, and patients with true red are often accompanied by an increase in white blood cells and platelets, which also increases the risk of blood clots in the patient. &nbsp.What are the ways to treat true redness? Commonly used drugs for the treatment of true red are hydroxyurea, alkylating agents, interferon, homoharringtonine and so on. In addition to western medicine, patients can also choose Chinese medicine treatment, intravenous bloodletting, and red blood cell apheresis. The administration of hydroxyurea is relatively common. Let’s learn about the pathological problems of this drug in the treatment of true red. How does hydroxyurea treat true red? Let’s first understand the incidence of this disease. True red patients are usually accompanied by JAK2 gene mutations. What is the JAK2 gene? ——JAK2 gene belongs to the JAK gene. STAT is phosphorylated and enters the nucleus to regulate the gene expression of the human body. This is the JAK-STAT pathway. After the patient’s JAK2 gene mutation, it will continue to activate the JAK-STAT pathway, leading to abnormal cell proliferation, apoptosis, and ultimately true redness. Hydroxyurea is a nucleoside diphosphate reductase inhibitor that can inhibit the reduction of RNA to DNA, and selectively hinder DAN synthesis, interfering with purine and pyrimidine base biosynthesis. At the same time, hydroxyurea also has an inhibitory function on bone marrow hematopoiesis, can effectively inhibit the production of red blood cells, platelets, and white blood cells, and play a role in treating true red. In the course of treatment with hydroxyurea, side effects such as vomiting, hair loss, and black nails will occur. Patients can choose symptomatic treatment to relieve the side effects. In addition, patients can also check relevant information in advance to learn as much as possible about the condition, so as to recover from the condition as soon as possible. For more patient communication help, please follow the WeChat public account [True Red Patient Club] zkxy120

Afraid of high platelets, what are the ways to lower platelets?

Thrombocytosis means that the platelet count in the patient’s peripheral blood is higher than normal. Not only that, patients may also form blood clots and spontaneous bleeding. For older patients with poor physical fitness, when bleeding and thrombosis occur, these will shorten the patient’s survival time to a certain extent, so the enthusiasm for treatment of this disease is emphasized. What methods are used to treat thrombocythemia clinically? The common ones are as follows: 1. Patients can use drugs such as hydroxyurea, interferon, and Marilan to inhibit bone marrow hematopoiesis, reduce the proliferation of hematopoietic stem cells, and reduce platelet counts. 2. Anticoagulant drugs such as aspirin and anagrelide can effectively inhibit platelet aggregation in patients, thereby improving microcirculation disorders and reducing the risk of thrombosis; 3. Blood cell apheresis is effective, rapid and effective through autologous blood collection and reinfusion. It can selectively reduce the number of platelets in the blood of patients; Fourth, patients with thrombocythemia are mostly accompanied by JAK2 gene mutations, so you can choose experimental drugs that target to inhibit JAK2V617F or MPLW515L/K signaling to improve the mutant JAK2 gene. To achieve the purpose of preventing thrombosis and reducing platelets. In Western medicine, the above-mentioned treatment methods are common methods that can help patients with thrombocythemia quickly control their condition. However, long-term medication is often required for patients, which means that patients have to bear certain side effects of drugs. Vigilance-patients are required to be prepared to alleviate side effects and take timely treatments that increase efficiency and reduce toxicity. For more patient communication help, please pay attention to the WeChat public account [thrombocytosis patients association] xxbzd999

What is the use of JAK2 genetic testing for thrombocytosis?

Patients with essential thrombocythemia often have symptoms such as dizziness, erythema, limb pain, and bleeding, and 50% to 80% of patients have splenomegaly. As the disease progresses, the patient will also form a blood clot, directly endangering the patient’s life. However, the diagnosis of thrombocytosis is not accurate based on these symptoms. Patients need to go to the hospital for standardized examinations and exclude other blood diseases before the diagnosis can be made. During the examination, the platelet count was too high, and the doctor ordered a JAK2 genetic test. What use is this? The incidence of JAK2 gene (V617F) mutation in MPD patients is very high. About 80% of PV, and 50% of ET and IMF patients have this mutation. JAK2 protein is a cytoplasmic tyrosine protein kinase that promotes the cascade amplification of intracellular signals. The V617F mutation of JAK2 gene causes the continuous phosphorylation and activation of JAK2 protein, which is believed to increase the sensitivity of erythropoietin and the survival of myeloid hematopoietic stem cells that do not depend on erythropoietin, which in turn leads to uncontrolled cell proliferation. JAK2 gene V617F mutation detection is a reliable method for differential diagnosis of MPD, which distinguishes MPD from other congenital or acquired polycythemia, platelet increase of unknown cause, and myelofibrosis of unknown origin. The JAK2 gene V617F mutation level varies greatly. It has been reported that the JAK2 gene mutation level is related to the symptoms and prognosis of the disease. After JAK2 genetic testing, it is helpful for doctors to formulate treatment plans that are more in line with patients. Most thrombocytosis is prone to relapse, so choosing the treatment that suits you is the most important. For more patient communication help, please pay attention to the WeChat public account [thrombocytosis patients association] xxbzd999

MPD Patient Association: What kind of gene is CALR? Will it affect the prognosis?

Gene mutations are often accompanied by myeloproliferative disease patients (but some patients show atypical negative conditions). The more prominent genetic abnormalities in this area are mainly related to JAK2 and CALR. The relevant analysis is carried out below. Is the prognosis of myeloproliferative disease (CALR gene mutation) good? Let’s first understand these two genes briefly-JAK2 gene mutations are not unfamiliar to everyone. It is more typical, and almost every patient will be tested for further diagnosis. It is not only helpful for early diagnosis, but also participates in an important part of the evaluation of the condition of patients with myeloproliferative diseases. As for the problem of CALR gene abnormality, according to clinical statistics, about 70% of JAK2 and MPL mutation-negative thrombocythemia and myelofibrosis patients have CALR mutations. However, it is relatively rare in patients with polycythemia vera. Many patients wonder what kind of gene CALR is? Will it affect the prognosis? To answer this-this gene is a mutation in exon ninth. In myeloproliferative diseases in China: myelofibrosis patients, the prognosis of CALR type 5bp insertion is worse than CALR type 52bp deletion (CALR gene mutation has these two Type) Fibrosis patients with CALR gene mutations have a lower incidence of red blood cell transfusion dependence and a higher survival time! Among patients with essential thrombocythemia in my country, patients with CALR mutations will have higher platelet counts than those with JAK2 mutations, but the values ​​of white blood cells, granulocytes, and hemoglobin will be relatively low. The conclusion is that the risk stratification is also low, so the prognosis is relatively good! Learn more about “disease knowledge” or “blood patient group communication” and other WeChat search public number: xejb120

Xiebie recorded medical words: What subsidy is for rocotinib for myeloproliferative diseases?

Rucotinib is a kinase inhibitor that can be used to treat myelofibrosis and polycythemia vera. The method of administration is simple, just take it orally. As an emerging drug, is there any special point about rucotinib in treating myelofibrosis? Shi Shurong/Xie Bielu Director WeChat consultation platform zkxk9999 is called “targeted drug” rucotinib, which mainly inhibits the activation of the entire JAK-STAT channel, reduces the abnormally enhanced signal of the channel, and obtains the therapeutic effect. The essence of JAK is a tyrosine kinase that binds to hematopoietic growth factor receptors in cells. As an inhibitor of the JAK2-STAT pathway, Rucotinib can specifically inhibit the abnormalities of the JAK2-STAT signaling pathway caused by the JAK2, CALR or W515L 3 gene mutations, thereby acting as targeted therapy, improving symptoms, and improving quality of life. purpose. So what is the price of rocotinib? On November 28, 2019, the 2019 China Medical Insurance Negotiation Drug Catalog was announced. Rucotinib (Jiekewei), as the world’s first drug for the treatment of myelofibrosis, was included in the reimbursement scope of China’s national medical insurance for the first time. The bid price of medical insurance is 5mg/tablet, 131.13 yuan. After Rucotinib was included in the medical insurance, the specific price fluctuated up and down. Regarding Lucretinib, there are related assistance programs: Jiekewei (Ruketinib) 1. Co-aid plan: every Jiekewei treatment year (12 months) as a cycle, patients provide the first 3 months of Jiekewei Drug certificate, 9 months after the assistance of Jiekewei drug after approval of the project. (Note: It is calculated as 30 days per month) 2. The first phase of the plan: For patients who meet the pre-sick subsistence allowance, free assistance will be provided after the project is approved. More and more clinical data show that rucotinib can improve the quality of life of patients, shrink the spleen, reduce blood transfusion dependence and improve the symptoms of myelofibrosis. Not only that, rucotinib can significantly extend the overall survival time of patients. However, patients are not allowed to take medicines privately. Patients with myeloproliferative diseases should take medicines safely according to the doctor’s instructions. For more patient communication help, please follow the WeChat public account [True Red Patient Club] zkxy120

MPD Patient Association: What does “triple negative” mean?

What is the “triple negative” in myeloproliferative diseases? This mainly involves the results of laboratory genetic data about this disease. There are three types: one, JAK2 two, CALR three, MPL and the above three gene mutations were tested negative for myeloproliferative patients as “triple negative”. Doctors said that JAK2 (V617F) plays an important role in the pathogenesis of thrombosis, and that CALR or MPL mutations and three gene mutations are negative can identify patients with a lower risk of bleeding. “Triple negative” patients may include patients with myeloproliferative diseases with non-classical MPL mutations, congenital thrombosis caused by JAK2, MPL or THP0 germline mutations, and patients with non-clonal abnormalities. Although the prognosis of these patients is generally good, in fact there are substantial differences. Thereby increasing the difficulty of clinical decision-making. Therefore, if you encounter difficulties in clinical diagnosis, you can seek help from higher-level hospitals for diagnosis and condition assessment. To learn more about disease knowledge or exchanges among myeloproliferative patients, you can search WeChat public account: xejb120

Why do you get polycythemia vera? Will it have a short lifetime?

Why do you get polycythemia vera? Will the survival period be short? …The first reaction of many true red patients when they hear a doctor’s diagnosis is usually the case. In particular, in the past, the health of the sick population will be more doubtful about the accuracy of the examination, the doctor’s diagnosis level, the hospital’s diagnosis process, and so on. The body is always healthy, why is it suddenly diagnosed as “polycythemia vera”? Studies have shown that 95% of “true red” patients have JAK2 gene acquired functional mutations, including JAK2V617F or JAK2 gene exon 12 mutations (K539L, N542-543del, E543-544del are the most common, mainly heterozygous mutations). JAK2 gene exon 12 mutations are found in 50%-80% of JAK2V617F mutation-negative PV patients. Compared with JAK2V617F positive PV patients, patients with mutations in exon 12 showed higher hemoglobin levels and low platelets and white blood cells. There was no significant difference in the risk of thrombosis, conversion to leukemia and secondary bone marrow fibrosis, and the incidence of death. In addition, it also includes: DNMT3a, ASXL1, TET2, IDH1/2 and other gene mutations. These mutations not only lead to abnormal proliferation of the erythroid system, but also the hyperplasia of the granulocyte and megakaryocytic system, and may constitute the molecular mechanism of PV progression to myelofibrosis and acute leukemia. This means that most of the “true red” patients have genetic mutations located in hematopoietic stem cells, which is the cause of the “culprit” of the disease. If you have any questions about this article or the disease, please feel free to follow us on WeChat Soyisou to learn more: xejb120

Explaining why JAK2 gene mutation causes “true red”? Shi Shurong explained

Polycythemia vera (PV), or “true red” for short, is a bone marrow proliferative tumor (MPN) that originates from hematopoietic stem cells. The main manifestation is a myeloproliferative tumor characterized by abnormally increased red blood cells and independent of the normal erythropoiesis regulation mechanism. Director Shi Shurong’s micro-signal xueyeke999JAK2 gene can be seen in almost all patients with true red. It is an important diagnosis. JAK2 (Janus kinase 2) is a member of the 4 members of the JAK family (TYK2, JAK1, JAK2, JAK3) and is a non-receptor Peptide kinase (PTK), located on the short arm of chromosome 9 (9p24), plays an important role in the regulation of hematopoiesis. JAK2 gene point mutation occurs in exon 14 of JAK2 gene 1849, that is, the base of codon 617 of JAK2 gene coding sequence is G-T transposed, so that the original guanine G is replaced by thymine T, The valine encoded by it becomes phenylalanine. JAK2V617F is a somatic function acquired mutation that occurs at the level of hematopoietic stem/progenitor cells. When JAK2V617F mutation occurs, even in the absence of EPO, it can cause the continuous activation and enhancement of JAK2 kinase and downstream signal transduction pathways, resulting in Inhibition of malignant cell proliferation and apoptosis eventually causes the occurrence of PV. In addition, JAK2V617F mutation can also induce activation and signaling abnormalities of thrombopoietin receptor (TPOR) and granulocyte colony stimulating factor receptor (G-CSFR), resulting in abnormal proliferation of megakaryocyte cell lines and granulocyte progenitor cells, ET and The incidence of PMF may also be related to this. JAK2 gene mutations are ubiquitous in PV patients, and JAK2V617F mutation-negative PV patients often have other types of mutations related to the JAK2 gene, especially JAK2 exon 12 mutations. In addition, it also includes: DNMT3a, ASXL1, TET2, IDH1/2 and other gene mutations. These mutations not only lead to abnormal proliferation of the erythroid system, but also abnormal proliferation of the granulocyte and megakaryocytic system, and may constitute the molecular mechanism of PV progression to myelofibrosis and acute leukemia. For more erythrocytosis disease knowledge or patient help, you can pay attention to WeChat public number: zkxy120

Shi Shurong’s appointment: What is the use of JAK2 genetic testing for patients with thrombocytosis?

The JAK2 gene is located on chromosome 9p24. If a gene mutation occurs, it is generally prone to some blood system diseases. For patients with thrombocytosis, whether it is to diagnose the disease or choose a treatment plan, JAK2 genetic testing has important significance. Director Shi Shurong’s micro-signal xueyeke999 clinical patients with primary thrombocythemia will have abnormally increased platelet values, splenomegaly, bleeding and thrombosis. Although it is a chronic disease, it will cause great harm to patients in the later stage. It is closely related to chronic myelogenous leukemia, polycythemia vera, and myelofibrosis, and is often collectively referred to as “myeloproliferative diseases”, which can be transformed into each other or occur together. The examination found that the platelet value was abnormally elevated, and patients diagnosed with primary thrombocythemia often require JAK2 genetic examination. What is the significance? The incidence of JAK2 gene (V617F) mutations in patients with primary thrombocytosis is very high, about 80% of PV, and 50% of ET and IMF patients contain this mutation. JAK2 protein is a cytoplasmic tyrosine protein kinase that promotes cascade amplification of intracellular signals. After the JAK2 gene undergoes V617F mutation, the JAK2 protein is continuously phosphorylated and activated, which is believed to increase the sensitivity of erythropoietin and the survival of myeloid hematopoietic stem cells that do not depend on erythropoietin, which leads to uncontrolled cell proliferation. The JAK2 gene V617F mutation detection is a reliable differential diagnosis method for MPD. It distinguishes MPD from other congenital or acquired polycythemia, unexplained platelet increase, and bone marrow fibrosis of unknown origin. The JAK2 gene V617F mutation level varies greatly. It has been reported that the JAK2 gene mutation level has a certain correlation with the symptoms and prognosis of the disease. In summary, JAK2 gene detection has very important guiding significance for treatment. When this gene mutation occurs, blood system diseases are prone to occur. Some clinically effective drugs can be used in clinical treatment. In addition, it must be prevented during the treatment process. Low thrombotic complications, clinical medicine should also be symptomatic. For more thrombocytosis disease knowledge or patient help, you can pay attention to WeChat public number: xxbzd999

There are as many as nine types of myeloproliferative diseases. This test is necessary!

Myeloproliferative disease (MPD) is a group of diseases caused by the continuous abnormal proliferation of bone marrow cells of a line or multiple lines, rather than an independent disease. Clinically, there are one or more abnormalities of blood cytoplasm and quantity, splenomegaly, bleeding tendency, and thrombosis. The cause of the disease in this group is unknown, and it is more common in the elderly. The incidence of these diseases in the epidemiological population is about 6-9 / 100,000 per year. There are as many as nine types of myeloproliferative diseases! This group of diseases is clinically divided according to the main cell series of hyperplasia: 1. Polycythemia vera (PV); 2. Primary thrombocythemia (ET); 3. Chronic idiopathic myelofibrosis (CIMF) ; 4. Chronic myelogenous leukemia (CML); 5. Chronic eosinophilic leukemia (CEL); 6. Hypereosinophilic syndrome (HES); 7. Systemic mastocytosis (SM); 8. Chronic neutrophilic leukemia (CNL); 9, 8P11 myeloproliferative syndrome (MPS); … The above briefly understands the range of diseases of myeloproliferative diseases. The following answers a frequently mentioned question: Do you want to spend money to check the JAK2 gene if you suspect myeloproliferative diseases? Medical experts said that the incidence of JAK2 gene (V617F) mutations in MPD patients is very high, about 80% of PV, and 50% of ET and IMF patients contain this mutation. JAK2 protein is a cytoplasmic tyrosine protein kinase that promotes cascade amplification of intracellular signals. After the JAK2 gene undergoes V617F mutation, the JAK2 protein is continuously phosphorylated and activated, which is believed to increase the sensitivity of erythropoietin and the survival of myeloid hematopoietic stem cells that do not depend on erythropoietin, which leads to uncontrolled cell proliferation. The JAK2 gene V617F mutation detection is a reliable differential diagnosis method for MPD. It distinguishes MPD from other congenital or acquired polycythemia, unexplained platelet increase, and bone marrow fibrosis of unknown origin. The JAK2 gene V617F mutation level varies greatly. It has been reported that the JAK2 gene mutation level has a certain correlation with the symptoms and prognosis of the disease. So this genetic test is necessary. If you have any questions about this article or the disease, please feel free to follow us on WeChat and search more about MPD patients: xejb120