I. Relevant epidemiology
1. Incidence rate: natural incidence rate is about 3.6-4.1/100,000 population.
2. Ethnic incidence pattern: High incidence in Caucasian populations in Northern and Western Europe, North America and Oceania, and low incidence in Asian ethnic groups.
3, age-related: males have a higher incidence rate than females in childhood, female incidence rate exceeds male incidence rate in middle age, male incidence rate increases again after age 50, and slightly dominates in old age.
4. Susceptibility-related factors: genetic factors; environmental factors.
II. Basic etiology
1.Genetic factors.
2.Ionizing radiation.
3.Drug factors.
4.Chemical factors.
5.Infection factors.
6.Food factors.
Third, the malignant hematopoietic clonal characteristics and pathogenesis of MDS
1, monoclonal.
2, low apoptosis.
3, abnormal differentiation function.
4, high drug resistance.
IV. Diagnostic conditions and typing of MDS
Diagnostic conditions.
1, clinical manifestations are mainly anemia, which may be accompanied by fever or bleeding.
2.Blood picture: complete blood cytopenia, or either or both lineage cytopenia, which may consist of giant red blood cells, giant platelets, nucleated red blood cells and other pathological hematopoiesis.
3. bone marrow picture: pathological hematopoiesis with three or two lines or any lineage of blood cells.
4. Excluding other diseases with pathological hematopoiesis, such as CML, MF, M6, ET, etc. and other red lineage proliferative diseases such as hemolysis, megaloblastic anemia, etc., excluding other allohematocritic diseases such as AA, PNH, etc.
V. Treatment of MDS
(A) Supportive therapy for MDS
1.EPO therapy: It is a better alternative therapy to blood transfusion.
Conditions: low serum EPO level before treatment (<100~200mu/ml), low percentage of primitive cells, low frequency of transfusion: better efficacy
Dosage: 10,000u~20,000u/d, daily or 3 times/week
Observation: If no response has occurred after 4-6 weeks of dosing, G-CSF, or GM-CSF should be used in combination, often doubling the response rate, especially for those with serum EPO <500mu/ml and with cyclic iron granulocytes.
Note: Iron supplementation, red systemic response usually after 2-3 months, if it still does not appear, it is considered a failure of therapy, gram terminate the treatment and change the method.
2, transfusion therapy: treatment of neutropenia: G-CSF/GM-CSF: reduce the rate of infection, does not affect the transformation of AML. Treatment of thrombocytopenia: TPO, IL-11, platelet transfusion.
(II) Low-intensity treatment for MDS
1.Immunosuppressive therapy: dexamethasone; ATG; cyclosporine A; arsenic
2.Molecular targeting therapy for MDS: FLT3 inhibitors; farnesyltransferase inhibitors: FTIs; anti-vascular neoplastics; response stop and its derivatives: response stop; ralidomide. Drugs targeting the VEGF receptor signaling pathway: DNA methylation inhibitors. Histone deacetylase inhibitors.
(iii) High-intensity therapy
1.Induction remission chemotherapy.
2.Hematopoietic stem cell transplantation.
Before choosing to perform intensive therapy, it is recommended to consider the following points.
1. For most patients with MDS, the only curative treatment is hematopoietic stem cell transplantation; however, SCT is indicated for only a small proportion of patients and is often accompanied by relatively high suppression-related complications and mortality.
2, Intensive combination chemotherapy can restore normal polyclonal hematopoiesis in some patients, but can only induce long-term disease-free survival in a very small number of patients, and most patients will relapse.
The natural course of MDS varies, and even in high-risk patients, the survival period varies from months to years.
4. Patients with multiple concurrent diseases, age, and other personal factors can affect the prognosis of MDS patients on SCT or intensive therapy.