Diagnosis and treatment of primary myelofibrosis in Chinese medicine

1. What is myelofibrosis (abbreviated as bone fibrillation)? Myelofibrosis often occurs in people in the 50-70 age group and is a myeloproliferative disease of unknown origin, which can be secondary to malignant blood diseases such as chronic granulocytic leukemia, true erythrocytosis, primary thrombocytosis, multiple myeloma, or bone tuberculosis, osteomyelitis, benzene poisoning, etc. The main manifestation is the proliferation of fibroblasts in bone marrow and deposition of collagen fibers, while the hematopoietic function of extramedullary hematopoietic organs such as liver and spleen is reactivated due to the inhibition of bone marrow hematopoietic function, resulting in the enlargement of liver and spleen, while the enlargement of spleen is more obvious. At present, bone fibrillation is still a difficult disease to treat, and traditional treatment can only improve the symptoms to a certain extent, but has no significant effect on prolonging life. 2. What are the symptoms of osteoarthritis? Many patients may be found to have a swollen spleen because of the swollen upper abdomen. Some studies have reported that the rate of spleen enlargement is about 25px per year, but there are individual differences. Because of the slow onset of the disease, 30% of patients may have no conscious symptoms at the time of physical examination and diagnosis, or may present only with weakness, excessive sweating, and wasting. Individuals with severe cases may present with severe pain in the left upper abdomen or left shoulder due to splenic infarction, and some may have bone pain, fever and bleeding. If the elderly do not care about the milder symptoms and do not seek medical attention in time, the condition may drag on too long with advanced anemia symptoms, pallor, or even hemolytic manifestations eye white mild jaundice, and swelling of both lower limbs may appear related to splenomegaly affecting venous blood return. 3. What is the most reliable test to diagnose bone fibre? In general, if a patient visits a specialist clinic, routine blood tests are the first step. Many patients may have normal or even slightly elevated hematocrit in the early stages; about 1/2-1/3 of patients show orthocytic hypochromic anemia, and reticulocytes may also be mildly elevated. By looking at the blood smear, teardrop-shaped, oval or target-shaped red blood cells can sometimes be seen, but this is not specific for bone fibrils. If the diagnosis is not clear from blood smears, bone marrow and biopsy should usually be given promptly, and this method is instructive as opposed to blood testing alone. The diagnosis is usually confirmed when increased bone marrow reticulocytes and collagen fibers (which may be absent in early stages), active proliferation of megakaryocytes and granulocytes and decreased erythropoiesis are seen. In addition, studies have found that 50% of patients have mutations in the JAK2 gene and 5%-10% of bone fibers have codon variants at MPL515, which can also help the diagnosis to some extent by examining chromosomal genes. 4. What is the role of splenectomy in the treatment of bone fibrils? Since most patients with bone fibrillation have significant enlargement of the spleen, the resulting discomfort and complications (e.g., dyspnea, swelling of the lower extremities, increased blood pressure) are among the reasons that prompt patients to wish to have their spleen removed for treatment. However, splenectomy is generally not clinically recommended for treatment. Splenectomy is also indicated in patients with severe comorbidities of giant spleen such as splenic infarction, but the risk of secondary hepatomegaly and thrombocytosis has been reported. In patients who cannot tolerate surgery but must reduce the symptoms of splenomegaly, especially in elderly patients, splenic radiotherapy may be used, for which allohematocrit is a major complication. 5. Is there a cure for bone fibrillation? The current view is that hematopoietic stem cell transplantation is the only possible cure for bone fibrillation, including allogeneic bone marrow and peripheral stem transplantation, generally allogeneic is the mainstay. Patients with acute myelofibrosis under the age of 40 should undergo bone marrow transplantation early. The disappearance of bone marrow fibrous tissue after successful partial transplantation is not affected by the degree of fibrous tissue hyperplasia, but transplantation-related toxicity should be fully considered. Patient age and health assessment are equally important influencing factors. Conventional treatments that improve the disease but do not shorten its course include hydroxyurea, androgens, prednisone, erythropoiesis-stimulating factor, and danazol. Low-dose thalidomide combined with prednisone has been reported to correct anemia and thrombocytopenia in 30-50% of patients. Imatinib is also being used, but its efficacy is limited. Currently, the relevant targeted therapeutic agent (JAK2 kinase inhibitor) is in the clinical trial stage and hopefully will have the expected efficacy and bring benefit to patients. 6. What is meant by “dry aspiration” of bone marrow and is it always possible to have “dry aspiration” of bone fibre? Clinically, many hematologic disorders require further bone marrow aspiration to confirm the diagnosis. If the bone marrow is not extracted during the process, it is called dry marrow aspiration. Many patients and their families may be dissatisfied and even complain because of this, thinking that it is caused by the poor technique of the doctor. In fact, it is uncommon that the bone marrow is not extracted because of poor technique, but more often because of obvious pathological changes in the bone marrow itself. Therefore, dry marrow aspiration is also a manifestation of disease, especially in myelofibrosis. However, it is not necessarily bone fibrillation, but also myelodysplastic syndrome, aplastic drinking blood, etc.; and bone fibrillation may not be dry in the early stage, which should be correctly recognized. Once dry aspiration occurs, the first consideration should be to change the puncture site, and bone marrow biopsy should be done in patients with bone fibrillation. 7. Is the occurrence of bone fibrillation genetically related? Since multiple members of a family have been found to have bone fibrillation, many people suspect that bone fibrillation can be inherited. The current view is that although there is a genetic chromosomal variation in bone fibrillation, it is mainly caused by environmental factors and is not usually passed on to offspring. However, it does not rule out the possibility that members of families with a history of bone fibrillation are susceptible to developing bone fibrillation, and that there is a genetic susceptibility. All in all, middle-aged patients should not be too concerned if they want to have children, but they should do frequent re-examinations during the pregnancy process.