Introduction:
Myelofibrosis(Myelofibrosis, MF) referred to as medullary fibrosis, is a kind of myeloproliferative disease caused by collagen hyperplasia in bone marrow hematopoietic tissue, and its fibrous tissue seriously affects hematopoietic function. Primary medullary fiber is also called "osteogenic sclerosing disease". "The unexplained myeloid metaplasia." The disease has different degrees of myelofibrosis, as well as extramedullary hematopoiesis mainly in the spleen, followed by liver and lymph nodes. The typical clinical manifestation is juvenile-erythrocytic anemia, and there are more teardrop-shaped red blood cells. The bone marrow puncture often shows dry pumping, the spleen is often swollen, and has different degrees of bone sclerosis.
Cause:
[cause]
It has not been elucidated that some scholars believe that bone marrow fibrosis is caused by abnormal stimulation of hematopoietic stem cells, leading to fibrous tissue hyperplasia and even new bone formation, and bone marrow hematopoietic tissue involvement eventually leads to hematopoietic failure.
The main pathology of MF was changed to myelofibrosis and extramedullary hematopoiesis in spleen and liver lymph nodes. The occurrence of myelofibrosis occurs from the center to the outer periphery, starting from the proximal epiphysis of the spine, ribs, pelvis and femur and tibia, and then gradually spreading to the distal end of the extremities.
1) Early whole blood cell proliferation with mild myelofibrosis bone marrow cells showed varying degrees of proliferation. Red, granular, and megakaryocyte cell lines all proliferated, with megakaryocytes being the most obvious. The fat vacuoles disappeared and the reticular fibers increased, but the normal structure of the bone marrow was not affected. Hematopoietic cells account for more than 70%, and bone marrow stroma is mainly composed of soluble collagen.
2) Metaphase bone marrow atrophy and fibrosis stage fibrous tissue hyperplasia, accounting for 40% to 60% of bone marrow, hematopoietic cells account for 30%, megakaryocytes still proliferate. The trabecular bone is increased and thickened, and new bone formation is formed adjacent to the bone marrow. Each of the scattered hematopoietic regions is separated by a parallel bundle or spiral of matter formed by reticular fibers, collagen fibers, plasma cells, and stromal cells.
3) End stage of advanced myelofibrosis and osteosclerosis. It is mainly composed of bone trabecular bone hyperplasia, accounting for 30% to 40% of bone marrow. Both fibrous and osteosclerotic tissues were significantly proliferated, and the medullary cavity was narrow. Except for megakaryocytes, the hematopoietic cells of other lines were significantly reduced. In this period, the bone marrow matrix component is mainly composed of polymeric proteins, mainly expressing fibronectin, and the distribution of exogenous protein and TENASCIN is increased.
symptom:
[clinical manifestations]
Most of the diseases are hidden and progress is slow. Many patients are often diagnosed after months or years of symptom onset. The most common symptoms are fatigue, weight loss and spleen compression. At first, the general condition is still good, and gradually the symptoms of spleen enlargement, hypermetabolism, and anemia are aggravated. In the advanced stage, there may be bleeding symptoms. Its clinical manifestations are mainly:
1) Spleen, hepatomegaly and splenomegaly are the most important clinical manifestations, and the incidence is almost 100%. Occasionally, the patient himself was found to have a lump in the left upper abdomen or was found during a physical examination. Some people think that the degree of spleen is related to the course of the disease. Every 1 CM under the spleen rib represents a one-year course. Due to the splenomegaly, the abdomen is often full or heavily stressed. The spleen touches solidly and generally has no tenderness; however, if the spleen grows too fast, local pain may occur due to local infarction of the spleen, and even a rubbing sound may be heard.
2) Most of the patients with systemic symptoms are fatigue, weight loss, heat, sweating and other symptoms. Appetite is generally or diminished. Late wasting is especially noticeable.
3) There is mild anemia in the early stage of anemia, which gradually worsens with the decrease of hemoglobin. The symptoms of pale, fatigue, weakness, shortness of breath after physical activity, and palpitations are obvious.
4) Early bleeding platelet count increased or normal, no bleeding symptoms. Late thrombocytopenia, the skin often has purpura or ecchymosis, may have nasal discharge.
5) Other minority patients may have unclear bone pain. Very few patients develop secondary gouty arthritis due to increased blood uric acid.
【Diagnostic criteria】
Domestic diagnostic criteria:
1, the spleen is obviously swollen;
2. Peripheral blood and nucleated red blood cells can be seen in the peripheral blood. There are different numbers of teardrop-like red blood cells. There may be red blood cells in the course of the disease. There are different numbers of teardrop-like red blood cells. There may be red blood cells, white blood cells and thrombocytopenia in the course of the disease. ;
3, bone marrow puncture multiple "dry pumping" or "hypoplasia";
4, pathological sections of spleen, liver and lymph nodes showed obvious proliferation of fibrous tissue;
The diagnostic IMF must have a fifth item plus any two of the remaining 4 items and can exclude secondary MF.
1. Blood: Hemoglobin is reduced, positive cells are positively pigmented anemia; white blood cells are more normal or increased, a few are reduced; platelets are reduced, and a small number of patients are significantly increased. See the classification of young particles, young red blood cells, basophils, teardrop-shaped red blood cells, polychromatic red blood cells. Reticulocytes increase (2% to 5%).
2. Bone marrow: bone marrow puncture is mostly dry pumping, bone marrow hyperplasia is mostly reduced, hematopoietic cells are significantly reduced, and non-hematopoietic cells can be increased.
3. Tissue biopsy: bone marrow: fibroblasts proliferate significantly. Liver and spleen: There is extramedullary hematopoietic foci.
4. X-ray examination: About 70% of patients have osteopetrosis; the trabecular bone boundary disappears and the appearance is frosted glass.
5. Genetic examination: Some patients have chromosomal abnormalities, but the Ph1 chromosome is negative.
Others: serum alkaline luciferase, lactate dehydrogenase, uric acid, vitamin B12 can be increased.
diagnosis:
The myelin should be differentiated from the following diseases: 1 Chronic myeloid leukemia: Both can have giant spleen, the number of white blood cells increases, and the peripheral blood appears granulocyte proliferation like neutrophils and late myelocytes, but the age of chronic granules is lighter. The white blood cell count is often more than 100,000/mm3, and there are fewer myelocytes in the blood. The deformity of red blood cells is not similar to that of myelin. Leukocyte alkaline phosphatase activity is reduced or eliminated and the ph' chromosome is distinguishable from myelin. 2 The disease must be distinguished from low-proliferative acute leukemia and other diseases that cause juvenile-erythrocytic anemia. Secondary myelin can be diagnosed from clinical manifestations or special examinations. Sometimes multiple sites, multiple bone marrow smears and biopsy are needed to exclude secondary myelin.
complication:
Common complications include various symptoms caused by spleen compression, anemia, and bleeding.
treatment:
【Conventional treatment】
Due to the insidious onset of myelofibrosis, the disease progresses slowly. In the early stage of the disease, if the symptoms are not obvious, and the anemia and splenomegaly are not serious, no special treatment or symptomatic treatment is generally required. The treatment of myelofibrosis should be chosen according to the condition and course of the disease.
1. Androgen can accelerate the maturation and release of red blood cells in the bone marrow, so that anemia can be alleviated. Generally, it takes more than 3 months to use drugs:
1 Kanglilong 2 ~ 4mg / time, 3 times a day, oral;
2 danazol 0.2mg / time, 3 times a day;
3 propionate propionate 50 ~ 100mg / time, daily or every other day, intramuscular injection.
2. Adrenal cortex hormone can inhibit the antigen-antibody reaction, reduce the destruction of red blood cells in the spleen or inhibit the immune destruction of red blood cells stimulated by immune complexes, and improve the permeability of capillaries. It can be applied to patients with hemolysis or hemorrhage. Generally, prednisone is used for 40-60 mg/day, and after 2 to 3 weeks, the amount of bleeding can be reduced or the number of blood transfusions can be reduced.
3, chemotherapy drugs have an inhibitory effect on bone marrow hematopoietic tissue, suitable for cases of spleen, white blood cells and platelet counts are too high. You can choose 2 to 4 mg / day of Malilan or 0.5 to 1.0 / day of hydroxy gland. Traditional Chinese medicine can alleviate the series of side effects of chemotherapy.
prevention:
First, prevention
Avoid contact with radiation and chemicals such as benzene and lead. Protective measures should be strictly implemented for occupational needs that are often exposed to these damaging factors. Daily life, diet and daily life should be regular, work and rest, diet should be moderate, especially pay attention to not eating too much frying, smoked, over-focus, rubber food, avoid, eliminate the impact of bad mood, keep optimistic, lively The psychological state, carry out appropriate sports activities, such as jogging, tai chi, etc. to smooth the blood and regulate the body and mind. If you suffer from chronic granules, osteomyelitis, bone tuberculosis, etc., you should be treated actively, patiently, persistently and regularly to prevent further development of the disease, especially the application of TCM syndrome differentiation to reduce the side effects of Western medicine and to regulate the body. Reduce secondary myelin.
Second, conditioning
(1) Life conditioning
Appropriately strengthen exercise and enhance physical fitness to reduce the chance of infection. Life is regular.
(two) diet conditioning
Strengthen nutrition, add more protein and various vitamins. Can be more appropriate to kidney, nourishing food, such as walnuts, red dates, peanuts and so on. Applicable to anemia, weakness and other symptoms and bone marrow suppression after chemotherapy. Source: Medical Education Network
1. Ginseng stewed lean red ginseng or American ginseng 10 grams, a little lean pork, add water 200ml, simmer for 2 hours, add a little salt to eat, make up the vitality. Applicable to people with obvious qi deficiency.
2. Black chicken stew: half a dry black chicken, 10 grams of wolfberry, add water 300ml, 2 slices of ginger, simmer for 2 hours, add a little salt to eat, nourish kidney yin, suitable for kidney yin deficiency.
3. Black bean mutton soup: a small black bean, 2 slices of ginger, 50 grams of mutton, simmer for 2 hours, add a little salt to eat. Wenyang kidney. Applicable to kidney yang deficiency.
4. Black bean soup: a small black bean, sauteed, 2 slices of ginger, 1 pond carp, washed, add 1000ml of water, simmer for 1 hour with a little salt. Warming kidney yang. Applicable to kidney yang deficiency.
(3) Spiritual conditioning
Maintain open-minded optimism, build confidence in the fight against disease, and cultivate a strong will.
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