What is myelodysplastic syndrome?

  Bone marrow is the site of blood production in the body and contains the seed cells for blood production, scientifically known as hematopoietic stem cells. When the bone marrow fails to produce blood properly due to disease, the body will manifest as anemia, thrombocytopenia and leukopenia. Myelodysplastic syndrome (MDS for short) is a group of diseases caused by abnormalities in bone marrow hematopoietic stem cells. Although the prognosis of this disease is poor, medical development has led to more and more treatment methods, and patients should cooperate with their doctors and actively cooperate with treatment.  1. What is myelodysplastic syndrome Currently, myelodysplastic syndrome is considered to be a hematopoietic dysfunction caused by abnormal proliferation and differentiation of hematopoietic stem cells, which is characterized by abnormal differentiation and development of myeloid cells in the bone marrow, and is characterized by a decrease in peripheral blood cells and pathological hematopoiesis in the bone marrow. The etiology of MDS is not clear, but it is speculated that it may be due to The cause of MDS is not clear, but it is thought to be a clonal proliferation of malignant cells caused by biological, chemical or physical factors such as genetic mutations and chromosomal abnormalities. Radiotherapy and chemotherapy of tumors are known triggers for the development of MDS, and when patients take chemotherapeutic agents or radiotherapy to treat potentially curable malignancies (e.g., breast or testicular cancer, Hodgkin’s lymphoma and non-Hodgkin’s lymphoma, etc.), they may be at increased risk of developing MDS. Myelodysplastic syndromes are mainly seen in the elderly, with a median age of onset of 65-70 years.  2. Symptoms of myelodysplastic syndrome Anemia is the most common symptom of myelodysplastic syndrome. MDS also often leads to a decrease in platelets, which play a hemostatic role in human blood, and patients with a significant decrease in platelets often develop bleeding spots, petechiae and petechiae on the skin, epistaxis, gum bleeding, and even life-threatening cerebral hemorrhage in severe cases. life-threatening brain hemorrhage may occur. Patients with myelodysplastic syndrome often have reduced white blood cells and decreased resistance, and are prone to recurrent fever due to various infections.  3. Diagnosis of myelodysplastic syndrome Myelodysplastic syndrome anemia is characterized by “macrocytic” anemia, and the mean red blood cell volume (MCV) is often greater than 100 fl in routine blood tests. If you have anemia with MCV greater than 100 fl, without folic acid or vitamin B12 deficiency, you should have a high suspicion of myelodysplastic syndrome and have a bone marrow aspiration to confirm the diagnosis. Bone marrow aspiration may sound scary, but it is actually not very invasive and is performed under local anesthesia with very little risk. With a bone marrow specimen, a variety of tests need to be performed. In addition to having an experienced bone marrow morphology examiner come in to observe under a microscope, a full range of cytochemical staining, iron staining, pathological sections, chromosomes, and fluorescence in situ hybridization (FISH) should be performed to provide sufficient information for diagnosis, staging, prognosis, and guidance for treatment.  The diagnosis of myelodysplastic syndrome can be considered in elderly people who present with macrocytic anemia, exclude megaloblastic anemia, and have bone marrow smears suggesting pathological hematopoiesis in the red and or granulocytic and or megakaryocytic lineages, which meet the diagnostic criteria for myelodysplastic syndrome. Confirmed myelodysplastic syndrome is classified into different types according to the lineage of hematocrit and myelopoietic hematopoiesis, the number of ringed iron granulocytes, the number of primitive cells in the bone marrow and chromosomal findings.  Based on the lineage and degree of hematocrit, the level of primocytosis and chromosomal alterations, patients with MDS are grouped into risk groups, which can be divided into relatively low-risk and relatively high-risk groups according to the score. The relative low-risk group is dominated by bone marrow failure manifestations, with slow disease progression and a low risk of conversion to acute leukemia. In contrast, the relative high-risk group has faster disease progression, a higher risk of transformation into acute leukemia and a shorter survival period.  4. Treatment of myelodysplastic syndrome (1) Treatment of patients in the relatively low-risk group Patients in this group have a low risk of metastatic leukemia, mainly manifesting as severe anemia and thrombocytopenia, and treatment is mainly to improve bone marrow hematopoiesis. High-dose recombinant erythropoietin, with or without granulocyte colony-stimulating factor, is usually the first choice of treatment, especially for patients with serum erythropoietin concentrations below 500 IU/L or with less than 2 units of red blood cells transfused each month. Moreover, erythropoietic drugs have no effect on the progression of acute myeloid leukemia. 5q-syndrome patients with a high response rate to ranadolamide may be treated with this drug as the first choice. In addition, some patients may be effective for immunosuppressive therapy, such as cyclosporine, anti-thymocyte immunoglobulin, especially in patients who are young, cytogenetically normal, with hypoproliferative bone marrow, HLA-DR15 positive or in the presence of paroxysmal sleep hemoglobinuria (PNH) clone. Demethylating agents (decitabine) may also be used in patients in the refractory relatively low-risk group.  (2) Treatment of patients in the relatively high-risk group Patients in this group are treated with chemotherapy to delay leukemic transformation due to the high risk of acute leukemic transformation. Demethylation therapy (decitabine) or combination chemotherapy for acute leukemia can be used. Due to the high side effects of chemotherapeutic drugs, it can lead to further decline of blood picture in a short period of time and increase the risk of serious infection, bleeding and organ complications such as heart, lung, liver and kidney in patients. Therefore, physicians need to thoroughly evaluate the patient’s condition and physical status and weigh the benefits and risks of chemotherapy to determine whether the patient can undergo chemotherapy. Patients with advanced age, poor general condition and poor organ reserve function can be treated with aggressive supportive therapy to improve their quality of life.  (3) Allogeneic hematopoietic stem cell transplantation This is the only possible cure for MDS. However, it is difficult to find a suitable donor because patients with MDS tend to be older, have more comorbidities, and have a higher risk of transplant-related complications and death. It is currently limited to a subset of patients who are young, in good general condition, and have a matched donor for allogeneic hematopoietic stem cell transplantation.  (4) Supportive therapy Myelodysplastic syndrome requires transfusion of red blood cells due to bone marrow failure, reduced whole blood cells, hemoglobin below 60 g/L or anemia symptoms such as obvious weakness, panic and chest tightness, and supportive therapy with platelet transfusion for patients with platelets below 20X109/L or with obvious bleeding tendency. The transfusion rate of elderly patients should not be too fast to avoid inducing heart failure.  (5) Iron removal therapy Compared to the low-risk group, patients have a longer survival period, and repeated transfusions can lead to “hemochromatosis”. Patients not only have darker skin, but also have excessive iron load deposited in all organs of the body, which can cause complications such as cardiac arrhythmia, heart failure, liver cirrhosis, diabetes, etc. The risk of infection increases significantly and the quality of life decreases. For patients with ferritin greater than 1000ug/L or transfusions that have exceeded 20-25U of red blood cells, combined depot iron therapy can improve the prognosis.  In conclusion, the natural course and prognosis of MDS patients are highly variable, and treatment needs to be individualized, and the treatment plan needs to be adjusted promptly according to changes in the condition during follow-up.  5.What to do if you have myelodysplastic syndrome Patients and their families are often worried about whether MDS is contagious, but in fact MDS is not contagious to relatives and will not be passed on to children. To prevent MDS, it is necessary to avoid exposure to radiation as much as possible, including frequent X-ray diagnosis and radiotherapy; avoid exposure to toxic chemicals and home decoration materials with excessive concentrations of benzene and formaldehyde. Pay attention to enhancing physical fitness, reasonable diet, and improving immunity, etc. In addition, the elderly should closely observe the aura signals of myelodysplastic syndrome. If a patient suddenly feels weak, walks with heavy legs, and finds that purple petechiae also appear on the arms, they should go to the hematology department of a regular hospital as soon as possible and do not delay treatment. It is not advisable to give up your life easily or to seek medical help indiscriminately and believe in any prescription.