Overview of myelofibrosis
Myelofibrosis, referred to as myelofibrosis, is a pathological state in which the hematopoietic tissue of bone marrow is replaced by fibrous tissue, affecting hematopoietic function, accompanied by extramedullary hematopoiesis in the spleen, liver and other organs. Myelofibrosis can be divided into primary and secondary myelofibrosis according to the etiology, and into acute and chronic types according to the progress of myelofibrosis. The majority of primary myelofibrosis is chronic, characterized by a slow onset, significant splenomegaly, the presence of juvenile granulocytes, juvenile erythrocytes and teardrop-like erythrocytes in peripheral blood, dry aspiration on bone marrow aspiration, more megakaryocytes and fewer other hematopoietic cells on bone marrow smear, and a bone marrow biopsy characterized by the presence of fibroblasts, fibroblasts, reticulocytes, collagen fibers and bone hyperplasia with a relative decrease in hematopoietic cells. There is extramedullary hematopoiesis in the spleen, liver and other extramedullary tissues. In contrast, secondary myelofibrosis is associated with definite disease, and the manifestations of extramedullary hematopoiesis and abnormal hematopoietic function are less severe.
Treatment of myelofibrosis
There is no effective treatment for myelofibrosis. Treatment should be based on the degree of myelofibrosis and clinical manifestations, with the aim of reducing symptoms and stopping the progression of myelofibrosis. The treatment aims to reduce the symptoms and stop the progression of myelofibrosis. It mainly includes correcting anemia, improving the hematopoietic function of bone marrow and relieving the compression symptoms caused by splenomegaly.
1.Correction of anemia
2.Chemotherapy
3.α-interferon
4.Thalidomide
5.1,25 dihydroxyvitamin D
6.Splenectomy
7.Spleen area irradiation
8.Bone marrow transplantation
Secondary myelofibrosis.
1, Etiology.
Secondary bone fibrosis is mostly reported as a case, the causal relationship between the primary disease and secondary myelofibrosis is unclear, and the pathogenesis is still unclear, presumably due to chemical, physical, infection, tumor, autoimmune disease, thyroid disease and abnormal immune regulation, secondary to abnormal hematopoietic stem cells, immune abnormalities, allergic constitution, metabolic abnormalities and other underlying pathologies, which may cause fibroblast proliferation through a variety of mechanisms, collagen synthesis increases.
2, Mechanism.
Many patients with bone fibrillation have immune abnormalities, commonly antinuclear antibodies, anti-smooth muscle antibodies, rheumatoid factor, positive Coombs test, positive lupus-like anticoagulant, increased immunoglobulins and circulating immune complexes, and the application of immunosuppressive agents such as prednisone may improve the disease, so some scholars speculate that the pathogenesis may be immune-related.
Clinical and laboratory studies have confirmed that vitamin D and the metabolites of parathyroid hormone can regulate the deposition of collagen in the bone marrow, thus when vitamin D metabolism lives parathyroid dysfunction, it can also lead to bone marrow fibrosis.
3.Clinical manifestations.
The clinical manifestations and signs of secondary bone fibrillation include the combined manifestations of the primary disease and bone fibrillation, such as the splenomegaly compression manifestation of myelofibrillation, hematopoietic disorders, extramedullary hematopoiesis, bone penetration dry aspiration, juvenile red and young granulocytic anemia, etc., in addition to the systemic and local manifestations of the primary disease, the clinical manifestations of the primary disease can appear earlier or can be more insidious.
4.Treatment.
The main thing is to clarify and treat the primary disease, which can make the symptoms and signs of myelofibrosis gradually remit in some patients, such as getting rid of harmful environment, treating tumor and immune diseases, controlling infection, etc. The treatment dose should be small to avoid side effects such as infection and bone marrow suppression.
5. Disease course and prognosis
Among myeloproliferative diseases, IMF has the worst prognosis. The duration of the disease varies, with a survival time of 1-20 years and an average survival time of less than 5 years. Most patients have progressive aggravation of splenomegaly, and about 8-20% of patients eventually evolve into acute leukemia, and those who turn to acute leukemia account for about 27% of the causes of death in myelofibrosis patients. Other causes of death are severe infections, hemorrhage, congestive heart failure, severe anemia, and systemic failure