refractory anemia
Introduction
Introduction Refractory anemia occurs mostly in elderly patients over the age of 50. The clinical manifestations are anemia, reticulocytes are reduced, and neutrophils and platelets in peripheral blood are mostly reduced. Primitive cells in the surrounding blood are rare, no more than 1%. Bone marrow cells proliferate normally or hyperplasia, erythroid hyperplasia is more obvious, ring-shaped iron granules are rare, and red blood cell morphology is more common. Granulocyte and megakaryocytic cells also have some morphological abnormalities, but are usually mild. The original cells in the bone marrow do not exceed 5%.
Cause
Cause
The MDS is original, meaning the cause is unknown. Or has a history of chemical carcinogens, alkylating agents or radiation exposure, ie secondary. Of all cases of acute leukemia, only a few patients were clinically aware of a clear MDS process. A special chromosomal abnormality can be seen in about 50% of patients with MDS. The mode of progression of MDS patients and their conversion to acute leukemia depends to a large extent on the type and amount of oncogene activated in the cell. It is currently believed that this disease occurs as a result of clonal variation in early hematopoietic stem cells and damage. Chromosome banding analysis and G6PD isoenzyme studies on bone marrow cells suggest that MDS is evolved from a stem cell and is therefore a clonal disease.
Examine
an examination
Related inspection
Serous effusion protein erythropoietin erythrocyte adenylate kinase alkaline spotting red blood cell count erythrocyte adenylate kinase
1. Anemia: Except for individual patients, most patients have different degrees of anemia symptoms as the main clinical manifestations, such as pale, dizziness, fatigue, shortness of breath after activity.
2, bleeding: more than half of the patients have bleeding, but the early bleeding symptoms are mild, mostly skin and mucous membrane bleeding, gum bleeding or nasal discharge, because it is not serious, rarely need special treatment, female patients also rarely have menorrhagia However, as the disease progresses to the advanced stage, the bleeding tendency is aggravated, and cerebral hemorrhage becomes one of the main causes of death.
3, infection: due to neutropenia and dysfunction caused by infection, early stage of the disease (refractory anemia, RA) is more stable, more serious infection and fever, late (RAEB or RAEBt) easier to concomitant infection. Due to low immunity, it is easy to cause latent infections and uncommon infections such as septic arthritis, tuberculosis, Pseudomonas aeruginosa conjunctivitis and gangrene. Mold infections are more common in the later stages, and sepsis is often the complication of the end stage of the disease and the main cause of death.
4, signs: some patients with liver, spleen, lymph nodes may have mild swelling, can appear at the same time, can also appear alone, because the degree is not significant and easily overlooked. A small number of patients may have sternal tenderness, ribs or joint pain in the limbs. The initial symptoms of MDS lack specificity, and some patients may have no obvious symptoms. Most patients have dizziness, fatigue, upper abdominal discomfort and joint pain. Most of them start with anemia and can be used as the first symptom of a visit, which lasts for several months to several years. About 20% to 60% of the cases have bleeding tendency in the course of the disease, the degree is different, and there are skin defects, bleeding gums and nasal discharge. The severe person may have digestive tract or cerebral hemorrhage. Bleeding is associated with thrombocytopenia, and some patients have defects in platelet function. About half of the patients have fever during the course of the disease, fever is associated with infection, heat type is variable, respiratory infections are the most, and the rest are sepsis, perianal, and perineal infections. In cases that have not been converted to acute leukemia, infection and/or bleeding is the leading cause of death. Liver and spleen may have moderate or mild swelling, and 1/3 of cases have lymphadenopathy, which is painless. Individual patients have sternal tenderness.
Diagnosis
Differential diagnosis
Pernicious anemia: Malignant anemia, also known as megaloblastic anemia, is rare in China. The cause is often caused by pregnancy, lactation, malabsorption of the stomach, malnutrition or oral folic acid antagonists (with antagonistic dihydrofolate synthetase to synthesize tetrahydrofolate). A drug that causes an obstacle, such as acetaminophen or methotrexate.
Severe anemia: The so-called anemia refers to hemoglobin in a unit volume of circulating blood, and the red blood cell count or hematocrit (pressure) is lower than the lower limit of the normal value. This normal value may vary depending on gender, age, altitude of living area, and changes in physiological plasma volume. The severity of anemia can be divided into four levels depending on the amount of hemoglobin. Severe anemia is hemoglobin 60g / liter ~ 31g / liter, has been flustered at rest.
Chronic anemia: Anemia associated with chronic infections, inflammation, and neoplastic disease, commonly referred to as chronic anemia (ACD), is one of the most common clinical syndromes. The onset of this disease is more insidious, more common in male middle-aged and older people, about 70% of cases are over 50 years old. Children are rare, but the incidence of adolescents has also increased in recent years. Diverse clinical manifestations, lack of special performance, often treated with anemia, bleeding and infection, some patients can be asymptomatic, found during the physical examination.
