polycythemia
Introduction
Introduction Erythroblasting refers to the fact that the number of red blood cells per unit volume of blood is higher than the normal limit. It is generally believed that adult men with red blood cells > 6 million / mm 3 and adult women > 5.5 million / mm 3 are erythrocytosis. Increased relative is seen in blood concentration, absolute increase seen in high altitude life, fetal and neonatal, severe physical labor, severe heart and lung disease and polycythemia vera.
Cause
Cause
1. Relative increase in plasma volume, resulting in a relatively increased red blood cell volume, seen in vomiting, diarrhea, excessive sweating, extensive burns, diabetes insipidus, adrenal insufficiency, thyroid crisis, diabetic ketoacidosis.
2. Absolute increase: (1) Secondary polycythemia is found in the high-altitude residents' medical education network, and the serious cardiopulmonary disease medical education network collects and organizes, abnormal oxygen hemoglobin disease with low oxygen carrying capacity, etc. Low oxygen saturation, resulting in increased compensatory erythropoietin, renal cancer, hepatocellular carcinoma, uterine fibroids, ovarian cancer, polycystic kidney, etc., erythropoietin non-compensatory increase, can also cause secondary Increased erythrocytosis.
(2) Polycythemia vera.
Examine
an examination
Related inspection
Erythrocyte volume distribution width (RDW) erythropoietin erythrocyte osmotic fragility test erythrocyte volume index erythrocyte life
The number of red blood cells and the amount of blood red and white in the unit volume of blood exceed the upper limit of the reference value. Generally, after repeated examinations, adult male red blood cells>6.0×10 12 /L, hemoglobin>17g/L, adult female red blood cells>5.5×10 12 /L, hemoglobin>16g/L is increased.
Diagnosis
Differential diagnosis
Polycythemia requires identification of the following symptoms.
1. Polycythemia Vera is a myeloproliferative disorder caused by the proliferation of abnormal pluripotent stem cell clones. The incidence rate is about 1/100,000, mostly in the elderly aged 60 or so. It is extremely rare in childhood, and only 1% of all cases occur under the age of 25. Most of the onset is slow. As a result of increased red blood cells, blood viscosity increases, blood flow is slow, microcirculatory disorders, systemic vasodilatation and congestion. Common symptoms include headache, dizziness, visual impairment, red complexion, conjunctival hyperemia, increased blood pressure, hepatosplenomegaly, and vascular embolism. It is also common in nasal discharge and skin freckle. Excessive sweating and weight loss at night are also common. Fundus examination showed retinal vein dilatation, congestion, thickness, dark purple and so on. About one-third of patients have diastolic blood pressure. Bone marrow cell chromosome examination can be seen in a variety of non-specific aberrations, such as the eighth tris, the ninth body or the 5 and 7 or 22 part of the deletion. The red blood cell count is mostly 6 to 10×10 12 /L (6 million to 10 million / mm 3 ) or more, hemoglobin 160 to 250 g / L (16 to 25 g / dl), hematocrit 54% to 80%, and white blood cells moderately increased. The thrombocytosis can reach 400 to 1100 × 109 / L (400,000 ~ 1.1 million / mm3). Myeloid hyperplasia is active and granulocytes/erythrocytes are decreased. Hemoglobin F is slightly elevated, and leukocyte alkaline phosphatase and plasma B12 are increased. Erythroid progenitor cells can be propagated in vitro without the need for erythropoietin. Arterial oxygen saturation was >92%.
Treatment methods are:
1. Venous bleeding can reduce blood volume to normal in a short period of time, reduce symptoms, reduce bleeding and thrombosis opportunities. Bleeding 200 to 400 ml every 2 to 3 days until the number of red blood cells is 6.0 × 10 12 /L or less, and the hematocrit is 50% or less. Bloodletting can maintain the effect for more than 1 month. This method is simple and can be used first. Younger patients, such as no thrombotic complications, can be treated with blood alone. However, after bloodletting, there is a possibility of causing an increase in the rebound of red blood cells and platelets. Repeated bloodletting has a tendency to aggravate iron deficiency, and it is advisable to pay attention. For elderly patients with cardiovascular disease, bloodletting should be cautious, once more than 200 ~ 300ml, the interval can be slightly extended. Blood cell separation can be a large number of red blood cells, but should be supplemented with a single volume of the same type of plasma, blood injection should be intravenous rehydration at the same time to dilute the blood.
2. Chemotherapy:
(1). Hydroxyurea is a ribonucleic acid reductase that has a good inhibitory effect on polycythemia vera, and has no side effects of leukemia. The daily dose is 15-20 mg/kg. If the white blood cells are maintained at 3.5 to 5 × 10 9 /L, the hydroxyurea can be intermittently applied for a long period of time.
(2). Alkylating agent? The efficiency is 80%-85%. Cyclophosphamide and L-aniline mustard (Malfaron) have a faster effect, and the period of remission is long-term with busulfan and chlorambucil. The curative effect lasts for about half a year. Nitrobutyric acid mustard has fewer side effects and is less prone to cause thrombocytopenia, which is an advantage. Alkylation agents also cause leukemia but are less radioactive. The amount and method of alkylating agent: starting dose of cyclophosphamide is 100-150 mg/d, busulfan, malfuron and chlorambucil are 4-6 mg/d, and can be maintained after 4 weeks of remission after remission. The dose, cyclophosphamide is 50 mg per day, and busulfan is 2 mg per day or every other day.
(3). The cephalosporin base? Domestic application of this product 2 ~ 4mg, added to the 10% glucose solution intravenously once a day, continuous or intermittent application of hematocrit and hemoglobin to normal. The average response time was 60d and the median remission period was over 18 months.
3. Interferon alpha treatment: Interferon inhibits cell proliferation. It has also been used in the treatment of this disease in recent years. The dose is 3 million U/m2, 3 times a week, subcutaneous injection. After 3 months of treatment, the spleen shrinks and the number of bloodletting decreases. The remission rate can reach 80%.
4. Radionuclide treatment of 32P -rays can inhibit nuclear division and reduce the number of cells. The initial oral dose was 11.1×10714.8×107Bq. After about 6 weeks, the number of red blood cells began to decrease, and the 3-4 months were close to normal. The symptoms were relieved, and about 75%-80% were effective. If the condition does not resolve after 3 months, it can be administered once more. The relief time is 2 to 3 years. 32P has the potential to convert patients into leukemia, so it has rarely been used in recent years. Poor prognosis, more death from venous embolism, major bleeding, or development of myelofibrosis and acute leukemia.
Second, familial benign polycythemia. Benignfamilial polycythemia is an autosomal hereditary disease with different penetrance. More rare. Mild symptoms, often headache, lethargy, dizziness and fatigue; or no symptoms at all. The sick child has a deep red complexion and conjunctival congestion, but no splenomegaly. Blood group examination only has excessive proliferation of red blood cell line, hemoglobin is often above 200g/L (20g/dl), and blood volume is increased. White blood cells and platelets are normal. There are the same patients in the family. This disease is mostly benign, and can live to normal years. If the blood is thick and the symptoms are caused, bloodletting therapy can be used.
Third, secondary polycythemia. Secondary polycythemia is caused by many different causes. A variety of diseases that can cause polycythemia: see the classification of secondary polycythemia:
1. Tissue hypoxia or oxygen release disorders
(1) Physiological:
1) fetal period
2) Insufficient oxygen content in the environment: plateau area
(2) Pathological:
1) Insufficient lung ventilation: lung disorders such as bronchiectasis, pulmonary heart disease, obesity (Pickwickian Syndrome)
2) Pulmonary artery and venous fistula.
3) Cyanotic congenital heart disease.
4) Abnormal hemoglobin disease: hemoglobin M, sulphi hemoglobin and methemoglobin have poor oxygen carrying capacity.
2. The function of bone marrow-forming red blood cells is enhanced.
(1) Endogenous:
1) Renal: nephroblastoma, adrenal adenoma, polycystic kidney, renal artery stenosis, etc.
2) Adrenal gland: pheochromocytoma, Cushing syndrome, congenital adrenal hyperplasia, adrenal adenoma and primary aldosteronism.
3) Liver: hepatoma.
4) Cerebellum: angioblastoma.
(2) Exogenous:
1) Apply testosterone or a similar drug.
2) Application of growth hormone.
3. Newborns.
(1) transfusion of the placenta: the mother transfused to the fetus, the recipient of the twins.
(2) The umbilical cord is ligated too late.
