pure red cell aplastic anemia
Introduction
Brief introduction of simple red blood cell aplastic anemia Pure red cell aplastic anemia (purered cellaplasia) is referred to as pure red aplastic anemia; it is a rare group of syndromes caused by selective redox cell series of red blood cell series. More than 100 cases have been reported in domestic literature. Most of the pathogenesis is related to autoimmunity. Clinically, it can be divided into two categories: congenital and acquired. Acquired can be divided into primary and secondary according to the cause, and divided into acute and chronic according to the course of disease. basic knowledge The proportion of illness: 0.001% Susceptible people: no specific people Mode of infection: non-infectious Complications: dizziness
Cause
Simple red blood cell aplastic anemia
Etiology: This disease is caused by a selective regeneration disorder of bone marrow red blood cells. The pathology of this disease is mainly due to the proliferation and differentiation of simple red blood cells, and the clinical anemia. With the reduction or lack of reticulocytes, the red blood cells in the bone marrow are significantly reduced, while the granulocyte and megakaryocyte lines are normal. Group disease. Recent studies have shown that pure red aplastic anemia is mainly an autoimmune disease. According to its etiology and pathogenesis, most scholars divide pure red aplastic anemia into: 1 congenital: seen in Dimand-Blakfan syndrome, autosomal recessive inheritance, often in the disease 2 to 2 years after birth; 2 acquired : Also divided into primary and secondary.
Prevention
Simple red blood cell aplastic anemia prevention
1, go to bed early and get up early, exercise the body. Insufficient sleep can reduce the body's immune function, and it is also easy to stimulate the fire, causing external injuries.
2. Keep your mind calm. Avoid anger in the spring, do not be too impatient, always keep your peace of mind.
3. Quit smoking, drink less and drink coffee. Smoking is the most vulnerable to damage to the respiratory surface barrier and induces disease onset. Tobacco, alcohol and coffee all stimulate nervous excitement. Some people want to "eliminate tension and fatigue", but actually weaken the body's disease resistance.
Complication
Simple red blood cell aplastic anemia complications Complications
The most common complication of this disease is developmental malformation.
Symptom
Simple red blood cell aplastic anemia symptoms common symptoms whole blood cells reduce hemolytic anemia
The common clinical manifestations of this disease are progressive severe anemia, normal red blood cell or mild macrocytic anemia, with significantly reduced or absent reticulocytes, normal or near normal white blood leukocytes and platelets; bone marrow nucleated cells Does not reduce, granulocyte and megakaryocyte series proliferation is normal, but the young red blood cell series is significantly reduced, or even completely lacking, individual disease can be seen that the young red blood cell series matures at an early stage, there are small clusters of primitive red blood cells with giant morphological changes, but Lack of more mature young red blood cells, iron kinetics, showed that its essence is erythropoiesis.
(1) There is no congenital pure red aplastic anemia (diamond-blackfan anemia): 90% of the onset from the first birth to the age of 1 year, rarely after 2 years of age, the genetic law is still unclear, there is familial, the child growth retardation A few also have mild congenital malformations, such as thumb deformity, and Fanconi anemia is rarely associated with malignant diseases. The number of erythroid progenitor cells is not limited, and the quality is abnormal, HbF increases, fetal membrane antigen i persists, and rescue Increased pathway enzyme activity indicates that the nucleic acid synthesis is defective. The lymphocytes of patients can inhibit the growth of normal erythroid progenitor cells in vitro, 20% of cases can be spontaneously relieved, 60% of patients are effective for adrenocortical hormone, and those who are ineffective can also do bone marrow transplantation.
(2) Acutely acquired pure red aplastic anemia: viral infection occurs in the course of chronic hemolytic anemia, especially human parvovirus B19 infection, optionally inhibiting erythroid progenitor cells, causing acute pure red aplastic anemia, Aplastic crisis called hemolytic anemia; in some cases, hematopoietic function temporarily stops after viral infection, resulting in a decrease in whole blood cells. Giant red blood cells appear in the bone marrow, also known as acute hematopoietic stagnation. Acute pure red aplastic anemia can also occur. In children aged 1-4 years, self-healing after a few weeks, there is no infection factor, said children with temporary erythropoiec disease, acute pure red aplastic anemia is also seen in viral hepatitis and certain drug-induced, such as phenytoin, azole Hemorrhoids, chloramphenicol, isoniazid and procainamide, etc., most cases will completely recover after stopping the drug.
(C) Chronic acquired pure red aplastic anemia: mainly seen in adults, 50% of patients with thymoma, only 5% of patients with thymoma have pure red aplastic anemia; most of these thymoma are benign, 70% are spindle cell type, a few It is malignant; women are more common (female: male is 3 to 4.5:1), and a few are still secondary to certain autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis, and certain tumors such as chronic lymphocytic leukemia. , chronic myeloid leukemia, lymphatic disease, immunoblastic lymphadenopathy, biliary adenocarcinoma, thyroid cancer, bronchial lung cancer and breast cancer, etc., unexplained patients called primary acquired pure red aplastic anemia, a variety of immune mechanisms caused by young Inhibition of erythropoiesis, anti-erythrocytic antibodies, anti-erythropoietin antibodies or inhibitory T lymphocytes in the serum of patients, patients often accompanied by a variety of immunological abnormalities such as increased or decreased immunoglobulin, monoclonal immunoglobulins and A variety of serum positive antibodies, such as cold agglutinin, cold hemolysin, heterophilic antibodies, anti-nuclear antibodies, etc., anti-human globulin test and other positive, individual patients may be associated with a variety of endocrine gland function reduction, no Thymoma PRCA more common in men (male: female was 2: 1).
Examine
Examination of simple red cell aplastic anemia
1, blood routine examination, X-ray examination, CT scan.
2, chronic type should be examined in detail for the presence or absence of thymoma, X-ray chest posterior position, lateral position and 20-degree oblique position, can detect 85% to 90% of thymoma, the detection rate of CT scan Can reach 100%.
Diagnosis
Diagnosis and diagnosis of simple red cell aplastic anemia
The disease should be distinguished from anemia caused by other causes.
Anemia caused by abnormalities in hematopoietic microenvironment
The hematopoietic microenvironment includes bone marrow stroma, stromal cells and cytokines.
1. Bone marrow stromal cells and stromal cells damaged by anemia, bone marrow necrosis, myelofibrosis, myelosclerosis, marble disease, bone marrow metastasis of various extramedullary neoplastic diseases, and various infections or non-infectious osteomyelitis Damage to the bone marrow stroma and stromal cells, abnormalities in the hematopoietic microenvironment and affect blood cell formation.
2. Anemia-derived stem cell factor (SCF), interleukin (IL), granule-mono-column colony-stimulating factor (GM-CSF), granulocyte colony-stimulating factor (G-CSF), erythropoietin (EPO), thrombopoietin (TPO), platelet growth factor (TGF), tumor necrosis factor (TNF) and interferon (IFN) all have positive and negative regulation of hematopoiesis. Insufficient EPO occurs in renal insufficiency, liver disease, and pituitary or hypothyroidism; neoplastic diseases or certain viral infections induce more hematopoietic negative regulators such as TNF, IFN, and inflammatory factors, which can cause chronic disease. Anemia (ACD).
Insufficient hematopoietic materials or anemia caused by obstacles
Hematopoietic raw materials are substances necessary for the proliferation, differentiation, and metabolism of hematopoietic cells, such as proteins, lipids, vitamins (folic acid, vitamin B12, etc.), and trace elements (iron, copper, zinc, etc.). Any deficiency or utilization of hematopoietic materials may result in reduced erythropoiesis.
1, folic acid or vitamin B12 deficiency or utilization of anemia caused by a variety of physiological or pathological factors caused by the body's folic acid or vitamin B12 absolute or relative lack of utilization or utilization disorders can cause megaloblastic anemia.
2, iron deficiency and iron utilization of anemia, which is the most common anemia in the clinic. Iron deficiency and iron utilization disorders affect heme synthesis, and this type of anemia is called hemoglobin synthesis abnormal anemia. The erythrocyte morphology of this type of anemia is small, and the central light-stained area is enlarged, belonging to small cell hypochromic anemia.
Hemolytic anemia (HA): red blood cells destroy excessive anemia.
Hemorrhagic anemia
According to the rate of blood loss, acute and chronic, chronic hemorrhagic anemia often combined with iron deficiency anemia. Can be divided into coagulopathy (such as idiopathic thrombocytopenic purpura, hemophilia and severe liver disease) and non-clotting diseases (such as trauma, tumors, tuberculosis, bronchiectasis, peptic ulcer, sputum and Two types of gynecological diseases, etc.).
