Do you really know about myeloproliferative diseases (tumors)?

The WHO 2008 revision of chronic myeloid neoplasms explicitly changed myeloproliferative disorders (MPD) to myeloproliferative neoplasms (MPN) a said neoplasm, which sounds a little scary. MPN is a group of diseases that originate from hematopoietic stem cells and characterized by excessive proliferation of one or more lineages of bone marrow (such as granulocyte system, red blood cell system, megakaryocyte system and mast cells). The WHO 2008 myeloproliferative neoplasms (MPN) include nine diseases: (1) chronic myeloid leukemia (CML) BCR-ABL positive; (2) true erythrocytosis (PV); (3) primary thrombocythemia (ET); (4) primary myelofibrosis (PMF); (5) chronic neutrophilic leukemia (CNL); (6) chronic eosinophilic leukemia-without additional classification (CEL, NOS); (7) hypereosinophilic syndrome; (8) mastocytosis (MC); (9) myeloproliferative neoplasm, unclassifiable (MPN, U). Li Dongyun, Department of Hematology and Oncology, Dongzhimen Hospital, Beijing University of Traditional Chinese Medicine (BCMU) And JAK2 gene mutation is clearly used as one diagnostic criterion for PV, ET and PMF; ET is diagnosed with a reduced platelet count of ≥450×109/L. However, generally MPN refers to true erythrocytosis (PV), primary thrombocytosis (ET) and primary myelofibrosis (PMF). The various diseases can be transformed into each other or exist in combination. Due to the excessive proliferation of blood cells in patients, they can lead to blood stasis and increased viscosity and become a high risk factor for various cardiovascular and cerebrovascular diseases. PV, early risk factors for ET are thromboembolic diseases, mainly myocardial infarction and cerebral infarction, followed by bleeding and intermittent claudication; late risk factors are mostly death due to severe anemia and heart failure caused by myelofibrosis, bleeding and recurrent infections, and some patients eventually transform into acute leukemia. In the past, MPN was thought to be acquired only by the elderly, but now it has become significantly younger, with many patients in their twenties, the youngest being only 11 years old, and with platelets that had reached more than 2000×109. In the early stage, there may be no symptoms, some patients have myocardial infarction, cerebral infarction and other vascular diseases, a routine blood count only to find high; and then look at the previous physical examination report, the blood count has long been high, but no discomfort simply did not care about it. Therefore, you should regularly check the blood routine, and when you find high blood cells, you must pay attention to it, go to the hematology department of a tertiary hospital, systematically do the relevant tests, and first except secondary hematocrit. True erythrocytosis (PV) is a condition in which the total number of red blood cells in the body is significantly higher than normal, commonly known as polycythemia vera. 30% of patients have bone marrow fibrosis, which eventually leads to bone marrow failure, and 10% of patients develop acute leukemia. Due to the increase in circulating red blood cells and blood viscosity, it can cause headache and dizziness, and even bleeding and thrombosis. There may be increased blood pressure and splenomegaly, and peripheral blood erythrocyte count, hemoglobin, and erythrocyte pressure product may be significantly elevated. Primary thrombocythemia (ET) is a significant increase in platelets with bleeding or thrombosis. The prevalence is 2:1 in men and women. 8% of patients develop myelofibrosis and 10% develop acute leukemia. The spleen may be enlarged and the peripheral blood platelet count may be consistently elevated, while platelet adhesion, aggregation and factor 3 function may be reduced. Primary myelofibrosis (PMF) Diffuse fibrous tissue hyperplasia of the bone marrow with extramedullary hematopoiesis, primarily in the spleen but also in the liver and lymph nodes. The disease can last for more than 10 years and has a progressively enlarged spleen, sometimes the whole abdomen is occupied by the spleen; there are infantile red blood cells, infantile granulocytes and teardrop-like red blood cells in the peripheral blood due to extramedullary hematopoiesis; the bone marrow is difficult to puncture due to fibrosis, and often no bone marrow can be obtained, i.e., “dry aspiration”; bone marrow biopsy can confirm the diagnosis. The cause of death is bone marrow failure or cardiovascular disease, and about 10% to 20% of patients eventually become acute leukemia. There is a lack of effective treatment for MPN, i.e., there are no methods and means of eradication or cure. The aim of treatment is to alleviate the disease, improve the quality of life, prolong survival and control disease progression. The hope: first, to reduce the risk of thrombotic complications, and second, to prevent progression to myelofibrosis and acute leukemia. Commonly used drugs include: interferon, hydroxyurea, thalidomide, and androgens, but the efficacy is often unsatisfactory. Traditional Chinese medicine considers MPN to be an internal blockage of stagnant blood. However, the causes of blood stasis vary, including Qi deficiency and blood stasis, Qi stagnation and blood stasis, phlegm and blood stasis, etc. Each patient needs individualized diagnosis and treatment. Combined with Chinese medicine, MPN treatment can improve systemic symptoms, prevent thrombosis, control the blood picture, reduce the dosage of western medicine, slow down the process of myelofibrosis, and partially reverse the degree of fibrosis, thus prolonging the survival of patients and improving their quality of life. Chinese herbal medicine can be used to treat each patient individually, which is more targeted. In addition, Chinese medicine can be taken orally, intravenously, and externally.