Polycythemia vera

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Clonal chronic myeloproliferative disease of hematopoietic stem cells
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Authenticity red blood cell Polycytosis (PV) is a Hematopoietic stem cells Of Clonality chronic Myeloproliferative disease PV starts insidiously and progresses slowly, usually going through the following two stages: ① proliferation or Erythrocytosis Erythrocytosis is often seen in the stage; ② Anaphase of erythrocytosis It shows pancytopenia Extramedullary hematopoiesis Hepatosplenomegaly hypersplenism and Myelofibrosis Bleeding and thrombus are the two main causes of PV clinical manifestation , a few patients can progress to acute leukemia
Foreign name
polycythemia vera
Visiting department
blood specialty
Common causes
Genetic susceptibility, possibly related to ionizing radiation, virus and other factors
common symptom
Bleeding and thrombosis
Chinese name
Polycythemia vera

clinical manifestation

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1. Non specific symptoms
include headache , weakness vertigo , fatigue tinnitus , dizziness, forgetfulness, etc Neurosis symptom. Heavy diplopia Blurred vision 5%~20% of PV patients can Gouty arthritis
Nearly 40% of patients will have skin itching, especially after scratching in warm water bath. PV patients often have digestive tract discomfort, mainly including upper abdominal discomfort, gastroscope Indicating stomach duodenum Erosion, etc., presumably related to Blood viscosity Increase gastric mucosa Abnormal blood supply.
It shows burning pain, whitening or Cyanosis , but the arterial pulse is normal.
four Thrombosis , embolism or Phlebitis
It is associated with significantly slow blood flow, especially with Thrombocytosis There may be thrombosis and infarction. Thrombosis is most common in limbs mesentery , Brain and Coronary vessel , paralysis symptoms may occur in severe cases.
Due to vascular congestion, intimal injury and platelet Factor 3 decreases clot There may be bleeding tendency due to poor retraction and other reasons. Most common in skin Ecchymosis Gingival bleeding , sometimes trauma or Postoperative bleeding More than that.

inspect

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hemoglobin ≥ 180g/L (male), ≥ 170g/L (female); Red blood cell count ≥ 6.5 × 10 twelve /L (male), ≥ 6.0 × 10 twelve /L (female). white blood cell Platelet count also increased to a certain extent.
2. Myelogram
The proliferation is obviously active, and granulocyte, erythroid and megakaryocyte lines are all proliferating, especially erythroid proliferation.
red blood cell Volume increase: 51Cr labeled red blood cell method: male>39ml/kg, female>27ml/kg.
Hematocrit increased: ≥ 55% in men and ≥ 50% in women.
Neutrophil alkaline phosphatase score increased>100 (none fever And infection).
Normal (≥ 92%), serum vitamin B twelve Increased (>666pmol/L).

diagnosis

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1. Clinical Polyemia Performance.
two Hemoglobin determination and Red blood cell count Significantly increased.
three Hematocrit Increase: male ≥ 0.54; Female ≥ 0.50.
4. Without infection and other reasons White blood cell count Multiple times>11.0 × 10 nine /L。
five platelet count More than 300 × 10 for many times nine /L。
six Myelogram It shows obvious and active hyperplasia Megakaryocyte The erythroid cells were all proliferated, especially the erythroid cells.
7. Exclusions Secondary polycythemia , especially because of the heart pulmonary insufficiency , kidney, liver cerebellum and Gynecological tumor And so on.

treatment

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The purpose of treatment is to make Blood volume And red blood cell volume close to normal, inhibit bone marrow Hematopoietic function So as to alleviate the disease and reduce complication
1. Venous bleeding
It can reduce the blood volume to normal in a short time, relieve symptoms, and reduce the chance of bleeding and thrombosis. Bleed 200-400ml every 2-3 days until Red blood cell count At 6.0 × 10 twelve /Below L, hematocrit is below 50%. One blood letting can maintain the curative effect for more than 1 month. This method is simple and can be used first. Younger patients without thrombotic complications can be treated by bloodletting alone. However, it is possible to increase the rebound of red blood cells and platelets after blood letting. Repeated blood letting also tends to aggravate iron deficiency, which should be paid more attention. For the elderly and patients with cardiovascular disease, blood letting should be cautious, and the blood letting should not exceed 200~300ml at a time, and the interval can be slightly extended. blood corpuscle A large number of red blood cells can be collected separately, but plasma of the same type with the same volume should be supplemented. When bleeding, intravenous fluid should be supplemented at the same time to dilute the blood.
(1) Hydroxyurea It is a ribonucleic acid reductase, which is good for polycythemia vera inhibition , without causing leukemia side reaction as White blood cell count Maintain at 3.5~5 × 10 nine /50. The hydroxyurea can be applied intermittently for a long time.
(2) Alkylating agent The effective rate is 80%~85%. cyclophosphamide And left-handed aniline acid Nitrogen mustard (Mafalun) has a rapid effect, Remission period Then use Bai Xiao'an and Chlorhexidine phenylbutyrate For a long time, the curative effect can last about half a year. There are few side effects of phenylbutyrate nitrogen mustard, which is not easy to cause Thrombocytopenia , is its advantage. Alkylating agents also have the risk of causing leukemia, but Radionuclide Not many.
(3) Harringtonine It is reported in China that this product is added to 10% glucose solution Intravenous drip Once a day, continuously or intermittently until hematocrit and hemoglobin are reduced to normal. Achieve mitigation Time average 60 days, and the median remission period is more than 18 months.
three Interferon alpha treatment
interferon yes Suppressor cell The effect of proliferation has also been used for the treatment of this disease in recent years. subcutaneous injection After 3 months of treatment, the spleen shrank and the number of bleeding decreased. Remission rate Up to 80%.
4. Radionuclide therapy
32P Beta ray Can inhibit cells Mitosis , reducing the number of cells. After about 6 weeks, the number of red blood cells began to decline, and it was close to normal after 3 to 4 months. The symptoms were relieved, and about 75% to 80% of patients were effective. If the disease is not relieved after 3 months Administration once. The remission time was 2-3 years. 32P has the risk of transforming patients into leukemia, so it has been rarely used in recent years.
(1) Secondary gouty arthritis Take allopurinol and indomethacin for treatment.
(2) Pruritus Oral cyproheptadine, cismin or Cimetidine
(3) Ischemic manifestations For those with limb or cerebral ischemia, short-term anti platelet aggregation Drugs, such as aspirin Pan Shengding