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polycythemia vera myeloproliferative


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Polycythemia vera into 1 case of myelodysplastic syndrome
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Patients, male, 60 .1997 with dizziness due to red face, blood showed WBC 13.5 × 109 / L, Hb 192 g / L, PLT555 × 109 / L. Bone marrow smear showed: hyperplasia of bone marrow cells was active, ratio of 1.31 grains of red, diagnosed as "polycythemia" (PV). after oral administration of hydroxyurea therapy has been regularly review the blood, the blood of three lines or less normal blood .2005 August found that three lines have different a decline in mid-.9 fever, diarrhea, pelvic pain, then income homes. check: T37.8 ℃, moderate anemia appearance, superficial lymph node enlargement no time. lung breath sounds thicker, not heard and rales . of: He Qi Shi Jun Zhang Xi Li Pu Right of: Shanghai Jiaotong University Affiliated Sixth People's Hospital Department of Hematology, Shanghai, 200233 Title: Clinical Hematology ISTIC English title: JOURNAL OF CLINICAL HEMATOLOGY, the volume (of ): 2007 20 (5) Classification: R551.3 Keywords: myelodysplastic syndrome marked true red blood cell hyperplasia Machine Key words: R55 R3 machine standard Key words: polycythemia vera bone marrow aspiration smear of blood into the superficial pelvic lymph node cell proliferation of three lines of pain lung breath sounds regularly reviewed hydroxyurea capsules are red oral treatment of anemia in patients with dizziness, diarrhea medical Income Fund: DOI: similar to the literature related to Notes
polycythemia vera myeloproliferative

Worry about the elderly polycythemia vera 4 (content expertise) to edit entries summary
There is no summary of the contents of the directory welcome to add the edit summary - [hide] 1 Overview 2 Etiology Epidemiology 3 4 5 pathogenesis of clinical laboratory 6 7 8 differential diagnosis of 9 treatment diagnosis prognosis 11 10 Edit this paragraph Prevention | Back to top Overview
Chinese name: old English name of polycythemia vera: senilepolycythaemiavera Alias: Old polycythemia vera; elderly polycythemia vera; senilepolycythemiavera
Polycythemia vera (polycythaemiavera) is a significant increase in red blood cells, characterized by cloning of unknown etiology, chronic myeloproliferative disorders. Its characteristics are total red blood cell count and total blood volume increased significantly, usually accompanied by an increase in neutrophils and platelets; increased blood viscosity; bone marrow hyperplasia, often with advanced bone marrow fibrosis and extramedullary hematopoiesis; spleen enlargement, skin and mucous membranes was unique red purple and a variety of vas
cular and neurological symptoms.
1. Polycythemia vera symptoms (PV) is currently considered a chronic clonal myeloid proliferative diseases. Although the absolute increase in PV is the red blood cell disease, but the serum EPO level is not high, even lower than normal.
2.PV diagnostic criteria so far is still not clear diagnosis of exclusion. Primarily to exclude secondary erythrocytosis sign (SP). Some scholars have suggested to identify the serum levels of EPO PV and SP. PV serum EPO levels are reduced or even absent, while the SP patients with normal or elevated. Determination of the level of EPO Some scholars believe that the diagnostic criteria should be used as one of PV.
3.PV as a clonal disease, large series of studies have shown that about 40% of patients newly diagnosed with chromosomal abnormalities del (20) (8.11), +8, +9. With the progress of recessive disease may also have del (1) P (11), del (3). Cloning of PV detection in patients with confirmed, the red blood cells, neutrophils, monocytes and platelets as compared with monoclonal and polyclonal lymphocyte TB, and SP for the polyclonal blood, so there should be signs Cloning cases the diagnostic criteria for PV. Edit this section | Back to the top of the epidemiology
Polycythemia vera by the Vaquez first described in 1892, and had the disease and the relative nature of a distinction between polycythemia. Osler (1903) and Rurk (1904), respectively, the clinical manifestations of this disease were added and in detail.
Scholars in China first reported in 1957. Polycythemia vera were more common in Caucasians, particularly common in Jews abroad to statistics of the disease incidence rate of 0.4/10 million to 1.6/10 million people. Male to female ratio of 2:1 or 1.2:1. Middle-aged, the elderly are more disease, 50 to 70 years is the peak incidence in recent years found that the average age at diagnosis of patients increased gradually. Authenticity of red blood cells group (PolycythemiaVeraStudyGroup; PVSG) reported cases, 6% had family history. Edit this section | Back to the top of the etiology
Unknown etiology. For the bone marrow to stimulate red blood cells produce too much oxygen; EPO (erythropoietin) the increase; red blood cells have extended life hypothesis was overthrown. Recent studies show that red blood cells do not extend life, but too much autonomy erythropoietin; this disease is the level of pluripotent stem cells in cloning disease. Patients with red blood cells, granulocytes and platelets contain only glucose -6- phosphate dehydrogenase (G-6-PD) isoenzymes-A and fiber cells and lymphocytes still contains (G-6-PD) with Engineering enzymes A, B type. That red blood cell, granulocyte and megakaryocyte originated from the same abnormal pluripotent stem cells. Proliferation of hematopoietic cells was possible mechanism as follows: â‘  uncontrolled proliferation of tumor stem cells; â‘¡ There is a proliferation of abnormal myeloid factors exist; â‘¢ stem cells on erythropoietin and other blood cell growth factor increased sensitivity. Have been reported, the patient's bone marrow and mononuclear cells in semi-solid culture, even in the absence of exogenous EPO under the conditions of a progenitor cell clone forming units (CFU-E) is called endogenous CFU-E . If added to the culture system is not only erythropoietin CFU-E increased, and in G-6-PD isoenzyme II A and B clones. That there are 2 kinds of normal and abnormal cell clones. It was envisaged that the bone marrow of normal stem cell cloning by an unknown mechanism inhibited, and cause abnormal cell clones continued proliferation, the generation of red blood cells can not be erythropoietin or red blood cell growth factor under the control and was independent of erythropoietin to increase, but also the formation of erythropoietin inhibit the normal red blood cells so that the Department of relative inactivity in addition, it was suggested that patients may be a factor in the stimulation of pluripotent stem cells and the formation of red directional effects. There are experiments show that erythroid progenitor cells in various culture system can spontaneously differentiate, growth, and is extraordinarily sensitive to erythropoietin, the anti-erythropoietin antibodies added to culture system inhibited erythroid colony formation but not clear . However, some people mentioned in the normal subjects and patients in cell culture dose response of erythropoietin shape of the curve not significantly different. Showed that the sensitivity of cells to erythropoietin without major changes. Edit this section | Back to the top of the pathogenesis
Pathogenesis has not yet fully understood. MeCulloch summary information on clonal proliferation of polycythemia that the abnormal clone has the following three characteristics: â‘  cell origin from a single, continuous proliferation; â‘¡ abnormal clonal suppression of normal clones have an advantage, the late disappearance of the normal clone; â‘¢ abnormal clone with cells genetic instability, clinical occasionally into the red to see real cases of acute leukemia. A similar phenomenon can be found in chronic myeloid leukemia, essential thrombocythemia and primary myelofibrosis with myeloid metaplasia, McCulloch called "clonal blood disease"; Edit this paragraph | Back to top laboratory
1. Blood was characteristic of dark blood, concentrated red corpuscular volume increased significantly, men ≥ 36ml/kg, women ≥ 32ml/kg (normal male 28.27 ± 4.11ml/kg, female 24.21 ± 2.59ml/kg chromium labeled red blood cell method .) About 2 / 3 cases of plasma volume below the lower limit of normal, no more than normal. Blood viscosity higher than normal, 5 to 8 times. Increase the proportion of blood up to 1.070 ~ 1.080 (normal 1.052 ~ 1.058). Arterial oxygen saturation ≥ 92% if> 92%, help with heart and lung disease caused by secondary polycythemia identification. Is worth noting that blood samples should be promptly inspected, in order to avoid spurious hypoxemia.
2. Blood when the first treatment, mostly in the red blood cell count (6 ~ 10) × 1012 / L, even as high (12 ~ 15) × 1012 / L; hemoglobin concentration up to (180 ~ 260) g / L (18.0 ~ 26.0 g / dl); hematocrit 0.60 ~ 0.80. Mild red blood cells vary in size, occasionally shaped red blood cells, or immature red blood cells. Reticulocyte numbers healthy. After repeated bleeding, increased reticulocytes. Erythroblastic easier to see, red states such as seen in iron deficiency anemia. Most of higher than normal white blood cell count to 25 × 109 / L or so common, few up to 50 × 109 / L or more, there are normal or reduced. Increase the number of granulocytes, a nuclear "left shift" phenomenon is sometimes seen in the neutral and the late promyelocytic promyelocytic cells, eosinophils and basophil increased; not seen the original granulocytes. Platelet counts often increased (internal data, about 2 / 5 cases have increased, foreign statistics, found in 3 / 5 4 / 5 cases), usually (300 ~ 1000) × 109 / L, individual    3000 × 109 / L can be seen, and abnormal giant platelets and megakaryocyte fragments.
3. Most of the bone marrow smear of bone marrow fluid examination showed a significant proliferation of hematopoietic cells in each department, the number of megakaryocytes increased volume change; a moderate increase in the percentage of nucleated red blood cells; granulocyte neutral and rods of late promyelocytic granulocyte common, eosinophil and basophil slightly increased. About 90% of patients before treatment, bone marrow cells in the blood inside and outside the dyeability of iron have been reduced or absent. Low bone marrow cell proliferation, can be found. In the bone marrow biopsy, 10% to 20% of cases early in the course of a net increase of hard protein and (or) fibrosis.
Barrlte living tissue such as bone marrow pathological changes proposed can be divided into four kinds: â‘  the classic three-line cell proliferation accompanied by large megakaryocytes, almost a lack of fat cells; â‘¡ red blood cells and two-line proliferation of megakaryocytes, giant cells clustered distribution of fat cells can be increased; â‘¢ two-line red blood cell and granulocyte atypical hyperplasia with few megakaryocytes; â‘£ Single - Department of proliferation of bone marrow red blood cells by electron microscope of the original immature red blood cell membrane of red early fold depth, nuclear membrane of neutrophil-like processes screen .
4. Modern research shows that part of the chromosome of patients without a variety of health often non-specific chromosomal aberrations, the most common abnormality 5q-, 20q-, C group attached chromosomes. The examination of the prognosis of a certain value.
5. Erythrocyte progenitor cells cultured in a variety of red blood progenitor cells can spontaneously differentiate culture system the growth of erythropoietin extremely sensitive, which required little or do not add to the formation of CFU-E. This test has diagnostic value.
6. Erythropoietin measured by radioimmunoassay, plasma and urine erythropoietin significantly reduced or absent. The results are different from the majority of secondary polycythemia.
7. Serum vitamin B12 and vitamin B12 binding capacity is not saturated in the health of patients without treatment, this two check values are increasing, but the two are not parallel, about 2 / 3 of the cases of unsaturated vitamin B12 binding capacity increased, and vitamin B12 content of normal this check will help with identification of secondary polycythemia, and can serve as indicators of the efficacy and disease activity.
8. Other ESR was slow. Most of neutrophil alkaline phosphatase points higher than normal. The amount of uric acid in serum and urine often increased. With nitrogen - urine uric acid glycine markers, patients with primary gout fast peaks are different. Life of most of the normal hemoglobin red blood cell production rate is about 2.5 times normal. Faster update rate of plasma iron. Bleeding and clotting time normal erythropoiesis proliferation cell nuclear antigen (PCNA) table in the detection, and response of the distinction between polycythemia. When the platelet count was significantly increased when there may be false hyperkalemia. Blood and urine histamine high-value are common. Other additional tests: basal metabolism slightly elevated. Edit this section | Back to the top of the clinical symptoms
Onset occult blood examination often even be found. Some cases in the event of a thrombosis and bleeding after the diagnosis. The main clinical manifestations are the following: 1. Vessels and the early performance of the nervous system may have headaches, dizziness, vertigo and tinnitus, fatigue, forgetfulness, numbness, sweating and so on. In severe cases, there may be blind spots, double vision and blurred vision and other visual anomalies. May also have angina pectoris and intermittent claudication. The group of symptoms is mainly due to increase the number of red blood cells, whole blood viscosity and increased blood volume increase caused by vascular dilation, stasis and slow blood flow caused by hypoxia. 2. Symptoms of thrombosis and embolism can occur in peripheral arteries, cerebral and coronary arteries, causing paralysis and myocardial infarction and other serious consequences. Thrombophlebitis with embolism occurs mainly in the lungs, but the mesentery, liver, spleen and portal vein may also occur, can cause acute abdomen. When associated with platelet count increased, the opportunity to increase the group symptoms. 3. Bleeding vessel congestion and vascular intimal injury and reduction of platelet factor 3 platelet dysfunction can lead to bleeding tendency. Common as nose bleeding, gum bleeding and petechiae on the skin and mucous membranes and petechiae and so on. 4. Histamine increased the performance of granular cells of the disease with increased health, basophil also increased, which is rich in histamine. Increase in histamine release can cause peptic ulcers, so patients with peptic ulcer of the higher than normal incidence of 4 to 5 times the upper gastrointestinal bleeding caused by ulcers can be life threatening. Skin itching is also common, occurring in the hot water bath 40%, 10% were associated with urticaria. The most common signs of bleeding caused by the face, nose, ears, lips and hands, and conjunctival congestion, dark red, such as drunk-like. Retina and the oral mucosa also show congestion. Arterial hypertension. About 3 / 4 of patients may have splenomegaly, secondary polycythemia because usually no splenomegaly, it has some signs of splenomegaly health significance of the differential diagnosis. Splenomegaly is not due to the increase in blood volume, so the blood does not reduce spleen treatment. About 1 / 3 of patients may have hepatomegaly, enlargement became clear with the development of the disease. Can occur in advanced liver cirrhosis and spleen enlargement situation not seen in about 10% of cases. Excessive congestion and extramedullary hematopoiesis may be the main cause splenomegaly. Complications: The most common concurrent bleeding, thrombosis, obstructive jaundice, biliary colic, myelofibrosis and leukemia. Edit this section | Back to the top of the diagnosis
Red roses with skin and mucous membranes, enlarged spleen, blood cells increased, especially in the performance of the three erythrocytosis and normal arterial oxygen saturation associated with the typical cases, diagnosis is not difficult. 1968 Polycythemia Vera Study Group diagnostic criteria proposed diagnostic criteria have been widely adopted as follows: â‘  The total capacity of red blood cells, male ≥ 36ml/kg health, women ≥ 32ml/kg (51Cr erythrocyte labeling method); â‘¡ arterial oxygen saturation ≥ 92%; â‘¢ enlarged spleen. Secondary indicators include: â‘  platelet count ≥ 400 × 109 / L; â‘¡ white blood cell count> 12 × 109 / L (no fever or infection); â‘¢ neutrophil alkaline phosphatase points> 100 (no fever or infection); â‘£ serum vitamin B12> 664.02pmol / L (900pg/ml), unsaturated vitamin B12 binding capacity> 1623.12pmol / L (2200pg/ml) who meet the diagnostic criteria in all three key indicators or comply with key indicators â‘ , â‘¡ item, plus any two secondary indicators established diagnosis. Determination of red blood cells if the total capacity being unconditional, refer to the 1971 proposed diagnostic criteria Modan: A. male hematocrit> 55%, women> 50%; B. no secondary or relative polycythemia a clear reason; C . any of various items of the following three, namely, white blood cell count> 12 × 109 / L (12000/mm3) (no fever, infection); platelet count> 300 × 109 / L (30   / mm3); arterial oxygen saturation was normal; neutrophil alkaline phosphatase points> 100; whole bone marrow hyperplasia, with increased megakaryocytes; enlarged spleen secondary erythrocytosis Zheng Jian on mountain sickness, a right-to-left shunt in congenital heart disease, chronic lung disease methemoglobinemia, the increase in oxygen affinity of hemoglobin disease, caused by smoking too much carboxyhemoglobin disorders, various tumors, especially liver, kidney, brain and uterine tumors, and hydronephrosis, renal artery stenosis, renal cysts, adrenocortical hyperfunction, and long-term use of male hormones. Sleep apnea syndrome associated with red blood cells can also be increased. Mostly the result of tissue hypoxia caused by increased erythropoietin, resulting in compensatory increase in red blood cells; erythropoietin, or EPO-like substance caused by an abnormal increase in polycythemia. Relativity and stress polycythemia Zheng Jian in severe dehydration, burns, adrenal insufficiency, etc., and neurotic obesity, mild obesity, healthy middle-aged patients. The most obvious reasons, systemic hypovolemia, is not difficult to identify with polycythemia vera. If hypovolemia is not difficult to identify with polycythemia vera health.
Edit this section | Back to the top of the differential diagnosis
If there is enlargement of the spleen and bone marrow blood cells increased three-line, but not enough red blood cells significantly increased the degree, you must consider early chronic myeloid leukemia or bone marrow fibrosis in early may. However, the early stages of chronic myeloid leukemia is characterized by mild erythrocytosis rather than anemia. In addition, 10% to 40% of patients have significant chronic myeloid leukemia, similar to the performance, so difficult to distinguish, detect Ph chromosome and neutrophil alkaline phosphatase activity can help identify. When the spleen is not enlarged, white blood cells and platelets do not increase the total capacity of red blood cells must be determined. Edit this section | Back to the top of the treatment
Treatment of this disease aims to enable the amount of red blood cells and whole blood volume close to or back to normal, so that in remission, reduce complications and prolong survival. 1. Venous blood method is simple, safe and effective rapid, short-term blood volume can be back to normal, reduce the risk of bleeding or thrombosis can be bled every 1 to 3 days 1 times, each time 300 ~ 500ml. Elderly and patients with bleeding associated with cardiovascular disease should be carefully each time should not exceed 200 ~ 300ml. Using blood cell separator can be a single mining a large number of red blood cells, but should complement and apheresis red blood cells and other volumes on behalf of the plasma or the same type of plasma red blood cell count dropped to 6 × 1012 / L (600   / mm3), hematocrit decreased to 50% testify to stop bleeding. Also that the hematocrit of 40% or less. Bleeding or blood clots can form after the complications of more loss of reduction is equivalent to 200mg per 500ml blood iron. Such as repeated blood without added iron, can cause small cell hypochromic erythrocytosis with iron deficiency performance. The absence of thrombotic complications in young patients, the bleeding can be a separate treatment to prevent long-term use of bone marrow suppression caused by drugs the possibility of leukemia and other malignancies. Reduce the bleeding just a product of erythroid hyperplasia, proliferation can not be suppressed, it can not eliminate pruritus, reduced hepatosplenomegaly, can not ease the platelet and leukocyte proliferation. But still high after a simple blood bleeding complications and thrombosis, it is like bloodletting at the same time in most patients with bone marrow suppression of drug treatment more affordable. 2. The most simple treatment of bone marrow suppression, Department of oral or intravenous 32P of the radionuclide concentration in the liver, spleen and bone marrow injected into the vein to avoid the shortcomings of irregular oral absorption. By 74 ~ 111MBq/m2 (2 ~ 3mCi/m2) Dose Intravenous administration. General often given intravenously after blood at the beginning of 32P111 ~ 185MBq (3 ~ 5mCi). 2 to 3 months after the blood, liver and spleen return to normal as possible reduced the proliferation of bone marrow is inhibited. The first injection of 12 to 16 weeks after the 2nd injection of 74 ~ 111MBq (2 ~ 3mCi) 75% ~ 85% of patients were satisfied effect. Remission for 6 to 24 months or more. Rarely occur after injection of bone marrow aplasia caused by the lack of granule cells and thrombocytopenia. 32P oral dose of 166.5 ~ 296MBq (4.5 ~ 8mCi) 2 times, fasting oral interval of 1 week. Before and after 10 days of food given to low phosphorus, to help drug uptake. For repeat treatment, two treatment rooms separated by at least four months. Retreatment compared with 1 dose should be reduced as appropriate dose. Cloned cells due to abnormal metabolism, so the 32P uptake more than normal cells. The 32P released by β-ray irradiation, inhibition of 32P nuclear fission and therapeutic purposes and for symptoms associated with elevated white blood cell and platelet counts in patients. Bleeding or thrombosis, bleeding after the use of 32P should be consolidated. Lower than normal white blood cells and platelets, severe liver and kidney disease of acute cerebral hemorrhage, active tuberculosis, pregnancy and lactation, secondary polycythemia, and relative contraindications are the 32P treatment. Long-term follow-up after treatment in patients with 32P, about 14% of patients with acute leukemia, is exposed to the same dose and time of the normal incidence of leukemia among the 20 to 40 times. Furthermore 32P treatment can also cause non-increase in the incidence of hematopoietic malignancies. This treatment applies only to need regular bleeding, long-term use are not effective inhibitors of bone marrow and liver and renal function in elderly patients is still good. 3. Chemical treatment is mainly used to splenomegaly compression symptoms, bone pain, liver enlargement was; require shorter time intervals bleeding; significant progress in the disease or past thrombosis and (or) vascular complications; blood after gastrointestinal discomfort with itching, weight loss, and there is embolism. Inhibition of hematopoiesis in the following types of drugs are available: (1) busulfan (Maryland): starting dose of 4 ~ 6mg / d maximum effect in 2 to 4 months later. About 84% of patients are expected to ease. Ease about the health of sustainable remission after 1 year as with 1 ~ 2mg / week maintenance therapy, medication for 4 Zhoujian Kang, intermittently for 4 weeks, remission can be extended to 3 years. When the platelet count ≤ 300 × 109 / L (30   / mm3) that should be discontinued. (2) chlorambucil: a starting dose of 4 ~ 10mg / d, maximum effect in 2 to 4 months later. 91% is expected, if not complete remission maintenance treatment, remission 5 to 6 months of chlorambucil mild side effects, remission rates are high, but also more opportunities for acute leukemia. (3) cyclophosphamide: a starting dose of 100 ~ 150mg / d to achieve complete remission for about 84 days, 87% remission is expected to ease from 5 to 6 months. (4) phenylalanine nitrogen mustard (Malta flange): starting dose of 4 ~ 6mg / d appears longer than the efficacy of busulfan (busulfan) is early. About 25% of patients can cause severe thrombocytopenia and lasted a long time, often difficult to control. Few other side effects, patients do not maintain acceptable if the amount of remission 6 months to 5 years. (5) cephalotaxine: China should be the FDA reported satisfactory outcome. 2 ~ 4mg plus 10% glucose solution intravenously, 1 / d, continuously or intermittently applied to the hemoglobin dropped to normal. That the drugs on DNA synthesis in erythroid inhibition, and some hematopoietic stem cells to kill or inhibit the average remission time of 60 days, median remission had more than 18 months the domestic treatment of 12 patients were in remission. 4. Splenectomy, some authors believe that splenectomy is a contraindication, as may occur after splenectomy thrombocytosis lethal. But there are of the view that fibrosis stage when the disease into the bone marrow, such as red blood cell life span was shorter, less severe anemia and thrombocytopenia, associated with splenomegaly caused by compression of gastrointestinal disease, splenectomy may be useful if the patient, especially those of bone marrow function better. If preoperative thrombocytosis may cause thrombosis and postoperative bleeding. After splenectomy the liver can be further enlarged. Operative mortality rate of 5% to 15%. 5. Interferon Cimino et al (1993) reported that treatment with interferon α-2b in 13 cases. Dose of 3.0 × 100   U health, intramuscular or subcutaneous injection 3 times a week. In the treatment of 6 months to obtain hematologic remission. The advantage for the non-induced leukemia induced thrombocytosis is not applicable to patients of different ages. 6. Other hyperuricemia gout, uric acid nephropathy, the use of allopurinol, 100 ~ 300mg / d, divided oral dose. Can also be treated with colchicine. Pruritus can cimetidine (Gimetidine) 0.3g, 3 times / d. Can also be used hydroxyurea. When severe itching and iron deficiency, after treatment with iron mechanism of itching will disappear is unknown. Bleeding times should be added due to iron deficiency. 7. Preferred program (1) for PV patients aged> 50 years old, accompanied by thrombosis, or platelets> 1000 × 109 / L were treated for bone marrow suppression. The drug of choice is hydroxyurea (HU). HU bone marrow as a non-alkylating agent by inhibiting ribonucleotide reductase inhibitors affect DNA synthesis, thereby inhibiting proliferation of bone marrow. Confirmed by clinical practice, the role of drug-free induced leukemia, it can be used as long-term safety of PR treatment. Usage: HU1.0 ~ 2.0g, 3 times / d orally. Attention to monitoring CBC, if the WBC fell to 4.0 × 109 / L will have to post-withdrawal increase of blood cells can be administered repeatedly. Maintenance dose given according to the specific circumstances, to ensure that white blood cells in the normal range, may be appropriate to use HU0.5g1 ~ 3 times / d. (2) TCM: Niuhuangjiedu films, there is bone marrow suppression in, but the toxic side effects, patients may have mild diarrhea, but can ease through the reduction, can be tolerated based on individual 2 to 4 tablets, 4 times / d, health can be long-term use (3) Patients who have certain economic conditions, available interferon α, (α-IFN) to control the proliferation of bone marrow PV of splenomegaly and skin itching also showed good results, especially in severe combined thrombosis better efficacy in patients. Early in the disease, Interferon-α can prevent the development of PV to myelofibrosis. Methods: Interferon α300U, 3 times per week subcutaneous treatment time to be 3 months or more, the sustainable economic conditions, medication. Repeat to disable treatment 1 month later. Injection may have a type of flu-like start performance: such as fever, joint and muscle pain. But after a few days disappear naturally. Plus indomethacin (indomethacin) or acetaminophen (paracetamol) may relieve adverse reactions. Edit this section | Back to the top of the prognosis
Natural history of slow progress of the disease 18 to 36 months. Without complications, a simple blood treatment, the median survival of 8 to 15 years. Cervantes and other reports, the expected 10-year survival 68.7% (48.1% ~ 89.3%). Leading cause of death is thrombosis, bleeding, myelofibrosis and leukemia. 5% ~ 15% of patients in the diagnosis of 5 to 13 years after the establishment of occurrence of bone marrow fibrosis and myeloid metaplasia and bone marrow biopsy Barrtle information such as proposed under the megakaryocyte more likely to develop bone marrow fibrosis type of the disease into the probability of leukemia treatment to some extent. Many data show that the use of any one kind of inhibitors of bone marrow after treatment of acute leukemia risk was increased and with the 2 or more types of drugs combined with, more dangerous. And should be carefully chosen. Edit this section | Back to the top of the Prevention
Because of unclear etiology of this disease, so their prevention, mainly in terms of early diagnosis and early treatment.
1. Note that the early detection of risk factors: the end of the regular purple face and limbs of patients, not to mistaken for emotional facial telangiectasia, or for the frequent occurrence of dizziness, headache, dizziness, limb numbness and other neurological symptoms and do not simply considered hypertension or cerebral insufficiency, if not improve upon the system governing the patients have to check the blood count, blood in order to increase early detection of abnormal red blood cells.
2. For patients who have been to ensure that reasonable treatment to prevent serious complications. Have occurred for the arterial and venous thrombosis and infarction in patients with major organ to control the disease, strengthening organ function recovery, the patient's quality of life improved. Edit entry Open Category:
Term evaluation of disease: participation in evaluating the authority of a total of 0 0% 0% rich professional good 0% 0% 0% I have to evaluate the poor:
● Introduction
Polycythemia vera (Polarthenua Vera, PV), also known as primary polycythemia, is an unexplained, chronic progressive bone marrow disease activity in general hyperthyroidism, bone marrow dysplasia is neoplastic disease. Red blood cell disease other than the week more than the normal blood-based, hemoglobin, hematocrit also increase accordingly. Clinical manifestations of the skin, mucous membranes purple, enlarged spleen, accompanied by thrombosis and neurological symptoms, white blood cells and platelets are often increased. The disease is less common, the domestic self-reported from 1957 to 1985, a total of 376 cases, the onset age of 31 to 60 years old mostly male sub women, and to older males. Natural course of disease is generally 18 to 36 months, if no complications, duration of up to 10 to 20 years, survival has been reported for more than 30 years. Course of the development is divided into three: â‘  increase of red blood cells and hemoglobin, the course for several years; â‘¡ of bone marrow fibrosis, the compensation of blood in the normal phase; â‘¢ anemia of shows splenomegaly, myeloid metaplasia, and whole blood cells reduced. Small number of cases can be transformed into acute leukemia.
The cause of polycythemia vera has not yet been fully elucidated, the detailed pathogenesis is also unknown. Red blood cell system, a significant proliferation may be related to the following factors: hematopoietic stem cell proliferation control, there does not depend erythropoietin erythroid clones showed hyperplasia; erythroid progenitor cells to erythropoietin in response to enhanced; serum there is an abnormal bone marrow stimulating factor.
Ancient Chinese medicine is no "polycythemia" disease name records, according to the four clinics see patients, the modern Chinese scholars to the category of "Vertigo," "Blood", "Zhengjia" diseases such areas. For red blood cell count, absolute increase in total capacity of the blood, liver and spleen enlargement was considered to be in the medical "evidence" that the business of blood caused by too real. Back in "the coffin on the sea," which said: ". who is more than gas, gas Manxiong in   free, the interest rate is more than the blood red face . is often thought of their bodies large and anger then I do not know their disease." True red blood cell see more patients described here in the bosom of gas full. Bored, wheezing, facial redness, body weight stagflation large, irritability and other symptoms. Patients with the disease can still see the red skin red, hi cold blood Division evil hot heat, the performance of more than yang, such as the "Miraculous Pivot meridians," said: "Where is the attending venae red hot ." " Cable asked to be as big of yin and yang, "also said:" The yang is hot, yin is cold ", can be found polycythemia vera lip A, looking dark, purple-red or purple tongue, liver and spleen enlargement, bleeding, thrombosis, numbness or pain, such as performance, undoubtedly the blood stasis syndrome. Such as "Jinbian Chamber" said: "chest full of patients, withered lips, tongue, . to have bleeding green." Stasis in which its different parts, the main symptoms were diagnosed as different can be the "Zheng Jia," " stroke, "" Chest 'and so on. polycythemia vera patients can be dizziness, headache, red eyes, irritability, pulse a few strings and other hyperactivity, anger expression on inflammation, such as "Blood Diseases of the heart" said: "Let the water depression for the fire, the blood does not and the fire made for the anger, the fire . too much blood must cross the buccal surface of green swollen, red eyes and headache. "Based on the above understanding, the modern Chinese scholars believe that polycythemia vera is more evidence of liver blood stagnation, the blood stasis-oriented, both anger, business real blood heat, so often used blood circulation, detoxify, spilled into the Liver , Liver and yang and other treatment.
● pathogenesis
(1) stagnation of qi and blood stasis
If the emotional depression, leading to liver qi stagnation, poor blood; or Concerned course of time, blood does not and, can be caused by bleeding within the stop. Visible abdominal bleeding is heavy on plot block the course of time, pain has given Department, dull red, astringent pulse. As with Yan Song and "re-Fang Zheng Jia Yan Shi Ji accumulation of Health and the door," said: "like a worry, thinking, happiness, anger of the gas, no person who can not, after almost five internal organs are injured ., Chuan Ke No, is the left end and for the five product "in the Qing Jing, especially in the" system of accumulation of gas Jinbian wing plot chapter, "also said:" The stagnation of qi into the plot too, where the Concerned Yu Nu, no solution for a long time who more into this disease. "They were both factors that can lead to product Kiyoshi permit occur.
(B) of the folder liver qi stagnation and blood stasis real fire
Liver depression and the course of time, the gas and blood stasis are run of the heat of the fire; or liver yang fire, anger is met through red after red eyes on the inflammation, headache, dizziness; Stagnant Heat plot block the formation of each node are shown in the liver and spleen swelling; blood stasis, stasis of blood Wang Xing is forced hot-free work Nvxue. As the "Treatment of Class CD Vertigo", said: "Feng Shui is a dirty liver and gallbladder, the send-fire, the main rise of active, or mental and physical hyperactivity, or emotional stagnation . so head faint ringing in the ears, dizzy earthquake uncertain" . "Medical error correction Lin," also said: "No matter where Jieyou blood, invisible gas can not caking, agglomeration choosing the visible blood, blood clot exposure to cold the blood is suffering heat into the block." In short arising from the liver disease and blood stasis, qi Erzhi anger, stasis and increase the heat each node congestion, so the anger caused by a variety of bleeding folder card.
(C) of the blood stasis and qi stagnation and heat penetrating the blood
Factor in the body heat; or yin deficiency heat of the body; or eat Feigan spicy products, endogenous heat Yu; or cult poison attacks the human body can be caused Yun Yu in hot blood Grading the stopping, heat penetrating the blood, heat and thermal disturbance of mind forcing the emergence of a series of hot blood blood camp of the card, such as fever upset, Shenhun saying. Bleeding.
In short, the disease polycythemia vera located in the liver, the emotional internal injuries, internal and external exogenous evil cult poison or Erzhi stagnation, qi the course of time the heat of the fire, forcing the blood of Wang Xing; or the course of time cause blood stagnation stasis, stasis and increase the heat each node seen bleeding and blood stasis syndrome.
Diagnosis
● Diagnosis and differential diagnosis
(A) Clinical features
Slow onset of the disease, duration of disease varies, the symptoms varied, sometimes without obvious symptoms, physical examination found only in, or see mild fatigue, dizziness embolism, patients are often ignored.
(B) of the card features Hou
(1) fiery performance of the liver: visible headache, stretching, dizziness, tinnitus or numbness, redness may face drunken state.
(2) bleeding symptoms: skin bruising, epistaxis, bleed at the nose gear is more common, can still see the brain, uterus and intestinal bleeding and trauma, postoperative bleeding and so on.
(3) Integrated Card performance: hepatosplenomegaly, hard, painful root.
(4) can still see the "Chest", "stroke", and abdominal pain and other symptoms.
(5) weight loss occurs with advanced multi-fatigue, skin a measure, increased sweating, weight loss and other symptoms.
(C) the differential diagnosis
1 phlegm: polycythemia vera patients with stasis based performance certificate should be differentiated with the disease. Phlegm and blood stasis due to the performance of both complex and diverse. Headache were seen, Xiong Xie fullness, vertigo, paralysis, numbness, delirium and other symptoms. However, due to pain and often has men Tong phlegm or pain, accompanied by white slip or the thick and greasy tongue coating, and this card, headache, abdominal pain tends to be stabbing, take root, see the tongue has purple or scarlet, pale purple , skin a mistake and so on.
2 Expansion: true erythrocytosis mainly to liver and spleen enlargement and bulging should be differentiated from disease. The former represents intra-abdominal lump or swelling or pain, take root, accompanied by pain. Zeyi bulging belly swell like a drum, greenish yellow skin color, context is characterized by exposure.
3 fullness: fullness is the swelling of the liver in patients with Cypriot Ministry Weiwanxiashu not conscious, uncomfortable fullness of a disease and syndrome. Outside the invisible card can experience, regardless of severity, were not touch the block material. The majority of polycythemia vera can reach the liver and spleen enlargement, both flank pain or tingling.
● Western diagnosis and differential diagnosis
Diagnosis〗 〖
1 Clinical manifestations
(1) General situation: most of the slow onset, early symptoms was not obvious, or only found in the examination. Common fatigue, weakness, dizziness, stretching, and weight loss.
(2) Multi-blood symptoms: headache, stretching, dizziness, tinnitus, skin and mucous dark red to the lips, nose, ear, hand and conjunctiva most prominent appearance of the face such as the drunk, high blood pressure, left ventricular enlargement of the heart to obvious.
(3) bleeding symptoms: skin bruising, epistaxis, see more teeth bleed at the nose, can also be found internal bleeding, postoperative bleeding and so on.
(4) the performance of thrombosis: portal vein thrombosis, obliterans, cerebral thrombosis or myocardial infarction can be seen.
(5), hepatosplenomegaly: about 75% of the patients may have mild to moderate splenomegaly, quality hard; 1 / 2 to 1 / 3 of patients have hepatomegaly.
(6), gastrointestinal symptoms: ulcers, gastrointestinal bleeding.
(7) gout: 10% of patients may be associated with gout.
2 laboratory
(L) blood: hemoglobin and red blood cell count increased significantly, many hemoglobin> 180g / L (men) or> 170g / L (female). Red blood cell count ≥ 6.5 × 1012 / L (men) or ≥ 6.0 × 1012 / L (female). Increased hematocrit: male ≥ 0.54, women ≥ 0.50. No infection and other causes an increase in white blood cell count (> 11.0 × 109 / L). Platelet count increased (> 300 × 109 / L) O
(2) absolute increase in red cell volume: 51Cr labeled red blood cell by law or 99Tc labeled red blood cell method, showing the absolute increase in red blood cell volume (more than ten of the unit normal 2 standard deviations).
(3) bone marrow: Changcheng or apparent active hyperplasia, proliferation of all three lines, especially with erythroid hyperplasia. Late immature granulocyte neutral particles and rod-shaped cells increased mainly megakaryocyte more common and more for the middle board of megakaryocytes. Extramedullary and sideroblastic iron reduction. Bone marrow biopsy sections showed that the main three-line cell proliferation, but the red lines and more common megakaryocytic, fat cells are replaced by hematopoietic cells, reticular cells when combined increased bone marrow fibrosis. Proliferation of reticular fibers is proportional to the degree of myeloproliferative states. But the failure of almost were highly reticular fibers when moderate to severe hyperplasia, often has patchy distribution, the number of reticular fibers calm is inversely proportional to the degree of bone marrow hyperplasia, the proliferation of bone marrow often highly active mild to moderate proliferation of reticular fibers, and proliferation of low-density bone marrow fibrosis is associated with Si.
(4) chromosome: without radiation therapy or chemotherapy in patients with normal bone marrow cells most chromosomes, a few shows aneuploid, hyperdiploid, hypodiploid and polyploid and other non-specific changes to C group the most obvious, after radiotherapy or chemotherapy, the variation is even more apparent.
(5) peripheral blood neutrophil alkaline phosphatase (NAP) activity increased, points> 100.
(6) Other tests: serum vitamin B12, uric acid were increased, an increase of serum lysozyme, serum iron decreased. Erythrocyte aspartate aminotransferase, G6PD, hexokinase were significantly increased. Serum erythropoietin (EPO) level is not increased. Increased blood viscosity, for 5 to 8 times normal.
3, the diagnostic criteria
National diagnostic criteria: ① how hyperlipidemia clinical manifestations: skin, mucous membrane was dark red, especially in the cheeks, lips, eyes with film, as the palm of your hand, etc.; splenomegaly; high blood pressure, or the course of the disease had thrombosis . ② laboratory tests: hemoglobin and red blood cell count increased significantly, many times without pre-treatment hemoglobin ≥ 180g / L (men) or ≥ 170g / L (female), red blood cell count of ≥ 6. 5 × 1012 / L (men) or 6.0 × 1012 / L (women); absolute increase in red blood cell capacity: red blood cells by the standard 51Cr method 99Tc labeled red blood cell method, showing the absolute increase in red blood cell volume (more than ten of the unit normal 2 standard deviation); increased hematocrit: male ≥ 0.54, women ≥ 0.50; no infection, and many other causes white blood cell count> 11.0 × 109 / L; platelet count greater than 300 × 109 times / L. ; Peripheral blood neutrophil alkaline phosphatase (NAP) integration> 100; bone marrow showed marked hyperplasia of type active active tablets were red and megakaryocytic hyperplasia, especially as the red blood cell. ③ to secondary polycythemia, except such as high altitude polycythemia; chronic lung disease caused by an increase in red blood cells, including the Eye Sa syndrome (Ayerza'Syndrome); congenital heart disease, especially tetralogy quadruple sign; poor lung ventilation syndrome, such as the Pickwick syndrome; and abnormal hemoglobin disease (including genetic and acquired, the latter due to heavy smoking, the carboxyhemoglobin concentrations as high as 4% to 6.8% can be generated when the absolute polycythemia); some tumors, cysts and blood vessels caused by abnormal red blood cells increase, such as adrenal-like tumor, hepatocellular carcinoma, hydronephrosis, renal artery stenosis, renal cysts, uterine leiomyosarcoma, cerebellar hemangioblastoma, as well as family sex and the "benign" erythrocytosis. ④ except relative to polycythemia, as a result of profuse sweating, severe vomiting, diarrhea, shock causes a temporary increase in red blood cells; and chronic relative polycythemia, such as Gaisiboke syndrome. Diagnosis of polycythemia vera may have two methods, preferably A method, measuring red blood cell volume does unconditionally, then using B method.
A method: a class with any of the above two; plus two categories in the first and second, plus three types of the disease can be diagnosed.
Method B: with a class plus the first and second in the first two categories (standard to male multiple hemoglobin ≥ 200g / L, women ≥ 190g / L). In addition, still need to have the third to seventh in any of four; plus three and four for diagnosis of the disease.
〗 〖Differential diagnosis
A secondary polycythemia: the people living in highland areas, people suffering from chronic heart and lung disease, long-term smokers will enable a large number of hypoxia, erythropoietin to increase, leading to red blood cells, hemoglobin increased, liver, renal cell carcinoma, cerebellar neoplasms, uterine cancer, hydronephrosis, renal artery stenosis. Adrenocortical hyperfunction, long-term application of hormones to erythropoietin-like substances increased or similar causes erythrocytosis.
2 relative erythrocytosis: a result of dehydration, burns to a decrease in plasma volume caused by blood concentration, expressed as the normal capacity of the surrounding red blood cells the body hematocrit increased.
3 Stress polycythemia: seen nervousness, obesity, middle-aged patients with mild hypertension.
4 Other myeloproliferative disorders: polycythemia vera, chronic myelogenous leukemia, bone marrow fibrosis, and essential thrombocythemia are the property of myeloproliferative disorders, and the tendency of mutual transformation. One each for each disease-based Department of proliferation can be accompanied by the proliferation of other cell lines. Therefore, some clinical and laboratory indicators of cross phenomenon, especially early in the disease will become more prominent.
Dialectical therapy
● dialectical points
Evidence-based early stage of the disease, especially in the main blood stasis and qi stagnation, Zheng Jian complexion, dark purple lips, skin a mistake, Xiexia plot block, dull red or ecchymoses, or astringent pulse string; the course of disease and See I suffer vertigo, dizziness, headache, hypochondriac pain, irritability, liver and gallbladder belongs to rely on the signs of real fire; if delirium and coma, Nvxue, hematochezia or hematuria, the color red is the blood of the elephant camp belonging to the Heat. Polycythemia vera patients often serious complications and even become the main symptoms of the disease, such as blood stasis appeared numbness, or even appear in the meridians, the organs of the stroke syndrome; systolic or Blockage may occur Chest really sad performance, clinical certificate should be combined with these two dialectic. In patients with advanced, as the disease advances, qi gradually consumption, congestion even worse, there may be seen in the virtual lack of bleeding and the performance of the actual situation was mixed with the card.
● Treatment principles
The basic pathological changes of the disease within the bleeding stops, so should the blood circulation as the basic rule is, and always should be run through the disease. And see if the liver and gallbladder, H.-S., should the law with Liver Xiehuo; and qi stagnation qi pain are advised to meet; and heat penetrating the blood were advised of the law with the heat and cooling blood; and hepatosplenomegaly, hard with , the straight line with Endometriosis of the law; if a stroke, Chest, etc. and the debate should be the self-governance of disease; advanced disease is a false positive imaginary evil Permit, supplementation and should be used the method of attack.
Prognosis and prevention
● outcome and prognosis
The beginning of the disease, mainly to the performance of bleeding and liver heat, mostly empirical. But long after Empirical blood stasis due to blood stasis of yin and yang of human blood metaplasia, mixed with the actual situation may be gradually transformed into the card, the performance of a qi deficiency, blood deficiency, yin, yang of the different parts of Yin and Yang deficiency of qi and blood of patients and there can be relative, such as Qi and Yin Deficiency, blood deficiency and qi and yang and other damage.
Most of the longer course of the disease, its severity and prognosis depend on the severity of blood stasis, blood stasis heavier, the prognosis is poor; if less blood stasis, the prognosis is fair. Surface course of the disease appear as dark purple lips, malaise, fatigue, limb against the cold, sharp mind tingling, pulse late knot or yang deficiency and blood stasis performance; or there Jian astringent language, physical exercise fail, or even confusion, such as performance are Critical syndrome. Forced as a result of heat or blood stasis blood, the blood does not follow by the sudden large number of vomiting blood, blood in the stool embolism is also intractable card, the prognosis is poor.
● nursing
Zhu Huanz
he attention to emotional conditioning should keep their minds at ease, not eat spicy things, should be eating into the light. Impassioned case of depression, the impassioned of the fire or the emergence of fresh bruises Yu-fire combined anger of the card worse. Another caution should instruct patient daily to prevent infection evils, Yu and heat, heat penetrating the blood into the pericardium or hot, crowded conditions orifices to turn adversity.
● Prevention
Should instruct patient usually living with restraint, to maintain ease of mind, the right to treat the disease, and establish the confidence to overcome the disease, or injured by the impassioned easy to make the disease worse. Diet should be light, not eat spicy heat of the products to help. Disease to the late inclusion of the actual situation as the disease mostly, it should be to prevent fatigue Haoshang righteousness, or workers sense of evils worse after the complex. Article entry: sosyao | Editor: sosyao
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