stomatocytosis
Introduction
Introduction to oral polycythemia Stomatocytosis (ST) is a morphologically abnormal erythrocytosis. A small number of oral cells can also be seen in the peripheral blood of normal people, generally 3% to 5%. If it exceeds 5%, it can be considered abnormal. basic knowledge The proportion of illness: 0.001% Susceptible people: no specific population Mode of infection: non-infectious Complications: splenomegaly
Cause
Causes of Oral Polycythemia
Cause:
Primary findings in autosomal dominant hemolytic disease, secondary to acute alcoholism, cancer, cardiovascular disease, hepatobiliary disease or certain drugs.
Pathogenesis
The main lesion of oral red blood cells is a significant increase in intracellular sodium content, a slight decrease in potassium, and an increase in sodium permeability from the cells, allowing sodium to continuously infiltrate into the cells from outside the cell, although Na-K ATPase activity is significantly increased (than normal Increased 6 to 10 times), but still can not compensate for the internal leakage of sodium, the result of a large amount of sodium leakage is caused by cell edema, swelling, volume increase, while the compensatory enhancement of Na-K pump function increases the utilization of ATP. Increased glucose consumption, accumulation of lactic acid, normal enzymes in sugar metabolism, normal ATPase, and imbalance in the active transport of sodium and potassium by Na-K pumps (normally 3:2, HST can be as high as 26:1), and the cell membrane is permeable to ions. Molecular lesions of sexual changes are unknown. Recently, the content of stomatin (one of the 7 proteins in the membrane) was decreased, but the gene was normal, the pore size and charged charge of the ion channel were normal, and the membrane lipid content was normal or increased. Lipid The transformation is normal, the membrane protein electrophoresis is normal, but the phosphorylation of membrane contractile protein is abnormal. After the oral red blood cells are treated with dimethyl adipimidate in vitro, the ion permeability of the cell membrane can return to normal, and the intracellular separation Sub-content and water are corrected, membrane deformability is improved, morphology is restored, cell life is prolonged, and oral red blood cells are often detained in the spleen sinus due to poor deformability. In the acidic environment of the spleen sinus, the available ATP is reduced due to glucose deficiency. The permeability of the cells to sodium is further increased, and therefore, the destruction of red blood cells in the spleen is more than three times that of other parts.
Prevention
Oral polycythemia prevention
The patient pays attention to the conditioning mood, keeps the mood comfortable, avoids spicy food, and should enter a light diet. In order to prevent depression, seven emotions of fire or stagnation of fire and the emergence of blood stasis combined with liver fire card to aggravate the condition. In addition, patients should be careful to live, to prevent infection of external evils, stagnation and heat, hot into the camp blood or heat into the pericardium, lively and clear, so that the condition turned dangerous.
It is generally necessary to keep the patient's daily life, to maintain a good mood, to treat the disease correctly, and to establish confidence in the fight against the disease. Otherwise, the illness of the seven emotions may make the condition worse. Diet should be light, eat spicy and hot products. When the disease is in the middle and late stages, the condition is often mixed with the image of the virtual and the real. Therefore, it is necessary to prevent overwork and injury, or to reinforce the exogenous evils after labor.
Complication
Complications of oral polycythemia Complications, splenomegaly
The main complications are seen in hepatosplenomegaly, and aplastic anemia or hemolytic crisis can be found at the time of infection.
Symptom
Symptoms of Oral Polycythemia Common Symptoms Red blood cell jaundice complexion red
The degree of hemolysis of HST is different. The lighter one has only the increase of oral red blood cells and no signs of hemolysis. In the course of the disease, occasionally due to infection, the risk of aplastic anemia is found. It is usually found in the family investigation, and the severe one may have life-threatening hemolysis. The HST hemolytic weight of the same family members is also different. Most HST may have intermittent anemia and jaundice. Severe patients often have splenomegaly. Secondary erythrocytosis usually does not cause hemolysis. According to clinical manifestations, oral red blood cells in the peripheral blood. Increased (up to 10% to 50%) hereditary oral polycythemia has a family survey, and the diagnosis is generally no difficulty.
Examine
Examination of oral polycythemia
Peripheral blood
Anemia is generally mild, hemoglobin is rarely lower than 80 ~ 100g / L, most patients with moderate increase in reticulocytes (10% ~ 20%), increased MCV, decreased MCHC, normal or increased, up to 10% of oral red blood cells in the peripheral blood ~50%.
2. Serum bilirubin and haptoglobin levels can reflect the extent of hemolysis.
3. Increased osmotic fragility, occasionally normal, increased autolysis test, glucose or ATP can be partially corrected.
4. Red blood cell life is moderately shortened, and water in red blood cells is increased.
According to clinical manifestations, symptoms, signs to choose to do ECG, B-ultrasound, X-ray, CT, MRI, biochemical examination.
Diagnosis
Diagnosis and differentiation of oral polycythemia
Mainly identify hereditary oral polycythemia, Rh deficiency syndrome and secondary oral erythrocytosis, secondary oral erythrocytosis except for peripheral blood erythropoiesis, generally no hemolysis, with the characteristics of the corresponding disease and lack of family history, identification Not difficult.
Rh deficiency syndrome is a very rare autosomal recessive hereditary hemolytic disease with an incidence of 1 in 6 million. The main lesion is gene deletion or gene expression disorder, and Rh blood group antigen can be completely lacking. Can also be partially lacking, the main clinical manifestations of mild to moderate anemia, hemoglobin between 80 ~ 130g / L, reticulocytes often between 6% ~ 12%, MCV and MCHC normal, peripheral blood can be seen a lot of oral red blood cells and a small amount Spherical erythrocytes, increased osmotic fragility, positive autolysis test, glucose or ATP can be corrected, red blood cell life is shortened, membrane lipid and membrane protein are normal, and splenectomy can improve hemolysis.
