Erythrocytosis (polycythemia) is characterized by a significantly higher than normal number of red blood cells, hemoglobin, erythrocyte pressure volume, and total blood volume. The diagnosis is made in childhood when hemoglobin exceeds 160 g/L (16 g/dl), erythrocyte pressure is greater than 55% and the absolute value of red blood cell volume per kilogram of body weight exceeds 35 ml, excluding relative erythrocytosis occurring as a result of hemoconcentration due to acute dehydration or burns, for example. Erythrocytosis can be categorized into two main groups: primary and secondary. Primary, i.e., true erythrocytosis; secondary is mainly caused by tissue hypoxia.
Polycythemia is characterized by a significant increase in the number of red blood cells, hemoglobin, erythrocyte pressure, and total blood volume above the normal erythrocytosis
levels. The diagnosis is made in childhood when hemoglobin exceeds 160 g/L (16 g/dl) [1], erythrocyte pressure is greater than 55% and absolute erythrocyte volume exceeds 35 ml per kilogram of body weight, and relative erythrocytosis occurs to exclude hemoconcentration due to acute dehydration or burns, for example. The syndrome can be categorized into two main groups: primary and secondary. Primary, i.e., true erythrocytosis; secondary is caused mainly by tissue hypoxia.
Edit True erythrocytosis
True erythrocytosis (polycythemia vera) is a myeloproliferative disorder due to the clonal proliferation of abnormal pluripotent stem cells. The incidence is about 1/100,000, most often occurring in the elderly around 60 years of age, extremely rare in childhood, and occurring in less than 25 years of age accounts for only 1% of all cases. The onset of the disease is mostly slow. The increase in red blood cells leads to increased blood viscosity, slow blood flow, microcirculation disorders, and vasodilatation and congestion throughout the body. Common symptoms include headache and dizziness, visual disturbances, redness, conjunctival congestion, increased blood pressure, hepatosplenomegaly and vascular embolism. Epistaxis and skin petechiae are also common. Excessive night sweating and weight loss are also common. Funduscopic examination reveals retinal veins that are dilated, congested, of varying thickness, and dark purple in color. About 1/3 of the patients have increased diastolic blood pressure. Chromosomal examination of bone marrow cells showed a variety of non-specific aberrations, such as trisomy 8, trisomy 9, or 5 and 7 or 22 partial deletions. Most of the red blood cell counts are above 6 to 10 × 1012/L (6 to 10 million/mm3), hemoglobin 160 to 250 g/L (16 to 25 g/dl), erythrocyte pressure 54% to 80%, and leukocytes are moderately elevated. Thrombocytosis can reach 400 to 1100 × 109/L (400,000 to 1.1 million/mm3). Bone marrow hyperplasia is active with decreased granulocytes/erythrocytes. Hemoglobin F is mildly increased, and leukocyte alkaline phosphatase and plasma B12 are increased. Red lineage progenitor cells proliferate in vitro in culture without erythropoietin. Arterial oxygen saturation >92%. Treatment methods are: 1, venous bloodletting Can be in a relatively short period of time so that the blood volume down to normal, reduce symptoms, reduce bleeding and thrombosis machine erythrocytosis
will. Every 2-3d bloodletting 200-400ml, until the number of red blood cells in the 6.0 × 1012 / L below, red blood cell pressure in 50% below. Bloodletting once can maintain the efficacy for more than 1 month. This method is simple and can be used first. Younger patients without thrombotic complications can be treated with bloodletting alone. However, bloodletting may cause rebound increase in red blood cells and platelets, and repeated bloodletting has the tendency to aggravate iron deficiency, so it is advisable to pay attention to it. For the elderly and patients with cardiovascular disease, bloodletting should be cautious, not more than 200-300ml at a time, and the interval can be slightly extended. Blood cell separation can be single-collected a large number of red blood cells, but should be supplemented with single-collected equal volume of homogeneous plasma, bloodletting should be accompanied by intravenous rehydration, in order to dilute the blood. 2, chemotherapy: ? (1). Hydroxyurea ? Is a kind of ribonucleic acid reductase, on the true erythrocytosis has good inhibitory effect, and no leukemia side effects, the daily dose of 15-20mg/kg. if the white blood cells are maintained at 3.5-5 × 109 / L, can be a long-term intermittent application of hydroxyurea. ? (2). Alkylating agent ? Effective rate 80%～85%. Cyclophosphamide and levobenzoate nitrogen mustard (Mafaram) act faster, and the remission period is longer than that of levobenzoate and phenylbutyrate nitrogen mustard, and the efficacy lasts for about half a year. Nitrogen mustard phenylbutyrate has fewer side effects and is less likely to cause thrombocytopenia, which is its advantage. Alkylating agents also cause leukemia but less than radionuclides. The dosage and method of alkylating agents: the starting dose of cyclophosphamide is 100-150mg/d, leucovorin, mafaram and nitrogen mustard phenylbutyrate is 4-6mg/d, and after remission and discontinuation of the drug for 4 weeks, the maintenance dose can be given, cyclophosphamide is 50mg per day, and leucovorin and so on is 2mg per day or every other day. ? (3). Triptorelin? Domestic reports apply 2-4mg of this product in 10% dextrose solution for intravenous drip once a day, continuous or intermittent application until the hematocrit and hemoglobin drop to normal. The average time to achieve remission is 60d, and the median period of remission is more than 18 months. 3、Alpha interferon therapy: ? Interferon has the effect of inhibiting cell proliferation, and has also been used in the treatment of this disease in recent years, with a dose of 3 million U/m2, 3 times a week, subcutaneous injection. After 3 months of treatment, the spleen shrinks and the number of bloodletting is reduced. The remission rate can reach 80%. 4、Radionuclide therapy β-rays of 32P can inhibit cell nuclear division and reduce the number of cells. The initial oral dose is 11.1×107～14.8×107Bq, about 6 weeks after the red blood cell count began to decline, 3～4 months close to normal, symptoms are relieved, about 75%～80% effective. If there is no remission after 3 months, another dose may be given. The time of remission is up to 2～3 years.32P has the risk of transforming patients into leukemia, so it has been rarely applied in recent years. The prognosis is poor, mostly dying from venous embolism, hemorrhage, or developing myelofibrosis and acute leukemia.
Edit Benign familial polycythemia
1. Benign familial polycythemia (Benignfamilial polycythemia) is an autosomal disorder with different epitopes Erythrocytosis
sexuality. It is relatively rare. Symptoms are mild, often with headache, drowsiness, vertigo, and fatigue; or no symptoms at all. The child has a deep red color and conjunctival congestion, but most often there is no splenomegaly. Blood tests show only hyperplasia of the erythrocyte lineage, with hemoglobin often above 200 g/L (20 g/dl) and increased blood volume. White blood cells and platelets are normal. There is a family history of the same patient. This disease is mostly benign, can live to normal years if the blood is sticky and produce symptoms, then bloodletting therapy can be used. 2. Polycythemia vera is a myeloproliferative disorder caused by clonal proliferation of abnormal pluripotent stem cells. The incidence rate is about 1/100,000, mostly occurring in the elderly around 60 years of age. It is extremely rare in childhood, and occurs in only 1% of all cases under the age of 25 years. The onset of the disease is mostly slow. The increase in red blood cells leads to increased blood viscosity, slow blood flow, microcirculation disorders, and vasodilatation and congestion throughout the body. Common symptoms include headache and dizziness, visual disturbances, redness, conjunctival congestion, increased blood pressure, hepatosplenomegaly and vascular embolism. Epistaxis and skin petechiae are also common. Excessive night sweating and weight loss are also common. Funduscopic examination reveals retinal veins that are dilated, congested, of varying thickness, and dark purple in color. About 1/3 of patients have increased diastolic blood pressure. 3. Secondary polycythemia (Secondarypolycythemia) is caused by many different reasons. Various diseases that can cause erythrocytosis are listed below: Classification of secondary erythrocytosis: A. Tissue hypoxia or impaired oxygen release B. Enhanced bone marrow production of erythrocytes
Treatment
Most patients do not require treatment, and those with mild symptoms are treated symptomatically. Intravenous bloodletting is considered only when there is a significant increase in erythrocytes (e.g., hematocrit >0.60) and thrombosis or other complications are likely to occur.
Secondary erythrocythemia
Secondary polycythemia (SER) is caused by a number of different factors. Various diseases that can cause erythrocytosis are listed below: Classification of secondary erythrocytosis: 1. Tissue hypoxia or impaired oxygen release (1) Physiological: 1) Fetal period 2) Insufficient oxygen in the environment: highland areas Erythrocytosis
(2) Pathological: 1) Insufficient lung ventilation: pulmonary disorders, such as bronchodilatation, pulmonary heart disease, obesity (Pickwickian), and 2) Pulmonary disease, such as bronchodilatation, pulmonary heart disease, obesity. Syndrome) 2) pulmonary arteriovenous fistula 3) cyanotic congenital heart disease 4) abnormal hemoglobinopathies: hemoglobin M, hemoglobin sulfide and n-ferric hemoglobin with poor oxygen carrying capacity 2. Enhanced bone marrow erythropoiesis (1) endogenous: 1) renal: renal embryonal histiocytoma, adrenal adenomatoid tumors, polycystic kidneys, renal artery stenosis and so on 2) adrenal: pheochromocytoma, Cushing syndrome, congenital adrenal gland Syndrome, congenital adrenal hyperplasia, adrenal adenoma combined with primary aldosteronism, etc. 3) liver: hepatocellular carcinoma 4) cerebellum: angioblastoma (2) exogenous: 1) application of testosterone or similar drugs 2) application of growth hormone
Edit this section of the newborn
(1) blood transfusion through the placenta: the mother's transfusion of blood to the fetus, the recipients of the blood in the twins Erythrocythemia
(2) Late ligation of the umbilical cord Secondary erythrocytosis is mainly due to a compensatory increase in the secretion of erythropoietin as a result of tissue hypoxia; or due to the occurrence of benign or malignant tumors that can produce erythropoietin and to the administration of hormonal agents that increase the production of erythropoietin. In newborns, it can be caused by transplacental blood transfusion or late cord ligation. Symptoms vary in severity, depending on the primary cause. White blood cells and platelets tend to be normal except for erythrocytosis. Treatment of the primary disease is the mainstay of treatment. Erythrocytosis is a compensatory phenomenon and does not require treatment. Erythrocytosis can be cured naturally after eradication of the primary disease. If the erythrocyte pressure area exceeds 65%, the blood viscosity is extremely increased, and the blood should be intermittently bled from the vein and replaced with an equal amount of plasma or saline.
Edit paragraph treatment
Venous bloodletting
Venous bloodletting can make the blood volume down to normal in a relatively short period of time, reduce the symptoms, and reduce the chance of bleeding and thrombosis. Every 2~3d bloodletting 200~400ml, until the red blood cell count is below 6.0×1012/L and red blood cell pressure is below 50%. Bloodletting once can maintain the efficacy for more than 1 month. This method is simple and can be used first. Younger patients without thrombotic complications can be treated with bloodletting alone. However, bloodletting may cause rebound increase in red blood cells and platelets, and repeated bloodletting has the tendency to aggravate iron deficiency, so it is advisable to pay attention to it. For the elderly and patients with cardiovascular disease, bloodletting should be cautious, not more than 200-300ml at a time, and the interval can be slightly extended. Blood cell separation can be a single large number of red blood cells, but should be supplemented with a single volume of the same type of plasma, bloodletting should be at the same time intravenous rehydration, in order to dilute the blood.
Chemotherapy
(1), hydroxyurea? It is a kind of ribonucleic acid reductase, which has good inhibitory effect on true erythrocytosis and has no leukemia side effect, the daily dose is 15～20mg/kg. if the white blood cells are maintained at 3.5～5×109/L, hydroxyurea can be applied intermittently for a long period of time. (2), alkylating agent? Effective rate 80%～85%. Cyclophosphamide and nitrogen mustard levobenzoate (Mafaram) act faster, and the remission period is longer than that of levobenzoate and nitrogen mustard phenylbutyrate, and the efficacy lasts for about half a year. Nitrogen mustard phenylbutyrate has fewer side effects and is less likely to cause thrombocytopenia, which is its advantage. Alkylating agents also cause leukemia but less than radionuclides. The dosage and method of alkylating agents: the starting dose of cyclophosphamide is 100-150mg/d, leucovorin, mafaram and nitrogen mustard phenylbutyrate are 4-6mg/d, and the maintenance dose can be given after stopping the use of 4 weeks after the remission, cyclophosphamide is 50mg per day, and leucovorin and so on are 2mg per day or every other day. (3), triclopyr alkaloid: China domestic report applies the product of 2-4mg, added to 10% glucose solution. Intravenous drip once a day, continuous or intermittent application until the hematocrit and hemoglobin drop to normal. The average time to achieve remission was 60 d, with the median period of remission exceeding 18 months.
Alpha interferon therapy
Interferon has an inhibitory effect on cell proliferation, and has been used in the treatment of this disease in recent years, with a dose of 3 million U/m2, 3 times a week, subcutaneous injection. The spleen shrinks and the number of bloodletting decreases after 3 months of treatment. The remission rate can reach 80%.Radionuclide therapy
Beta rays from 32P inhibit cell nuclear division and reduce cell count. The initial oral dose is 11.1×107 to 14.8×107Bq, and the red blood cell count begins to decrease after about 6 weeks, approaching normal in 3 to 4 months, with some relief of symptoms, and is effective in about 75% to 80% of cases. If there is no remission after 3 months, another dose may be given. The time of remission is up to 2～3 years.32P has the risk of transforming patients into leukemia, so it has been rarely used in recent years. The prognosis is poor, mostly dying from venous embolism, hemorrhage, or developing myelofibrosis and acute leukemia.
Editor's introduction
Tan Tianpu Famous hematologist Tan Tianpu is a professor of the Hematology Center of the First Branch of the 309th Hospital of the People's Liberation Army (PLA), a supervisor of doctoral students, and the director of the Hematology Research Department, a member of the Third Discipline Evaluation Group of the Medical Degree Committee of Beijing, a member of the National Foundation for Medical Science in the initial evaluation and re-evaluation, and the Standing Committee Member of the Hematology Committee of the Chinese Medical Association (CMA). He received the first batch of government special allowance and the first batch of Beijing Science and Technology Top Talent Award.
Edit paragraph treatment principle
The basic pathological change of erythrocytosis is blood stasis, so it is appropriate to activate blood circulation and eliminate blood stasis as the basic treatment principle, and should be throughout the disease. If the liver and gallbladder fire, should be combined with the method of clearing the liver and diarrhea fire; with the stagnant qi should be combined with the movement of qi to relieve pain; with the heat into the blood, should be combined with the method of clearing the heat and cooling the blood; with the liver and spleen enlargement, hard, it is straight to cooperate with the method of softening and dispersing the knot; if there is a stroke, thoracic paralysis, etc. and should be according to the respective main symptom of the dialectic; the late patients belong to the evidence of the positive and the evil, should be used in the method of attacking and supplementing the evidence of the late-stage patients. Regression and prognosis: In the early stage of the disease, blood stasis and liver heat are the main manifestations, mostly solid evidence. However, after a long period of solid blood stasis, due to the stagnation of blood stasis affecting the production of qi, blood, yin and yang, the disease can gradually turn into a mixture of deficiency and solid evidence, the manifestation of deficiency has different qi, blood, yin and yang deficiencies, and part of the patient's qi, blood, yin and yang deficiencies can be concurrently, such as qi and yin deficiencies, qi and blood deficiencies and qi and Yang, etc. Most of the disease has a long course. Most of the patients have a long course of the disease, and the severity and prognosis of the disease depend on the severity of blood stasis; if blood stasis is more severe, the prognosis is worse; if blood stasis is less severe, the prognosis is still good. If there are manifestations of Yang deficiency and Blood stasis such as dull face and purple lips, tiredness and fatigue, coldness of the limbs, severe stabbing pain in the heart and chest, and knotted or delayed pulse in the course of the disease; or manifestations such as imprecision in speech and limb movement, or even delirium, etc., these manifestations are considered to be critical symptoms. If the blood is not circulating through the channels due to heat compelling the blood circulation or blood stasis obstruction, the sudden large amount of blood vomiting or blood in stool is also difficult to treat and the prognosis is poor. Nursing care: patients should be advised to pay attention to the regulation of emotions, keep a relaxed mood, avoid eating spicy things, and eat a light diet. In order to prevent the seven feelings of depression, seven feelings into fire or food depression into fire and the emergence of blood stasis combined with evidence of liver fire to aggravate the condition. In addition, patients should be advised to be careful in their daily life to prevent infection with external evils, which may cause heat to enter the camp and blood, or heat to enter the pericardium, and make the condition of the clear orifices become critical. Prevention: patients should be advised to live and work in moderation, keep a relaxed mood, treat the disease correctly, and establish confidence in overcoming the disease, otherwise the condition will be aggravated easily by the seven emotional injuries. Diet should be light, avoid eating spicy and hot products. Disease to the middle and late stages, the condition is more mixed phenomena, so we should prevent overwork exhaustion, or labor after the recurrence of foreign evil aggravate the condition.