What Are the Different Types of Polycythemia Vera Treatment?

Polycythemia vera (PV) is a clonal chronic myeloproliferative disease of hematopoietic stem cells. The onset of PV is insidious and progresses slowly, usually through the following two stages of progression: Proliferation or erythrocytosis often has erythrocytosis; In the later stage of erythrocytosis, whole blood cells decrease, extramedullary hematopoiesis, hepatosplenomegaly, and hypersplenism And bone marrow fibrosis. Bleeding and thrombosis are the two main clinical manifestations of PV, and a few patients can progress to acute leukemia.

Basic Information

English name

polycythemia vera

Visiting department

Hematology

Common causes

Genetic susceptibility, which may be related to factors such as ionizing radiation and viruses

Common symptoms

Bleeding and thrombosis

Clinical manifestations of polycythemia vera

Non-specific symptoms

Including headaches, weakness, dizziness, fatigue, tinnitus, vertigo, forgetfulness and other similar neurological symptoms. In severe cases, diplopia and blurred vision. Gouty arthritis can occur in 5% to 20% of PV patients.

2. Itchy skin and peptic ulcer

Approximately 40% of patients experience itchy skin, especially after scratching with warm water. Patients with PV often have gastrointestinal discomfort, including epigastric discomfort, gastroscopy suggesting gastroduodenal erosion, etc. It is speculated that it may be related to abnormal blood supply to the gastric mucosa due to increased blood viscosity.

3. Erythematous limb pain

Burning pain, whitening, or cyanosis at the extremities, but normal arterial pulsation.

4. Thrombosis, embolism or phlebitis

Associated with significantly slower blood flow, especially with thrombocytosis, thrombosis and infarction may occur. Thrombosis is most common in the limbs, mesentery, brain, and coronary vessels, with severe symptoms of paralysis.

5. bleeding tendency

Due to vascular congestion, endometrial damage, decreased platelet factor 3, poor blood clot retraction, etc., there may be bleeding tendency. It is most common in skin ecchymosis, bleeding gums, and sometimes visible trauma or bleeding after surgery.

Polycythemia vera

Blood image

Hemoglobin 180g / L (male), 170g / L (female); red blood cell count 6.5 × 10 ¹² / L (male), 6.0 × 10 ¹² / L (female). White blood cell and platelet counts also increased to some extent.

Bone marrow

Hyperplasia was significantly active, with granulosa, red, and megakaryocytes all proliferating, with the red line proliferating significantly.

Increased red blood cell capacity: 51Cr labeled red blood cell method: male> 39ml / kg, female> 27ml / kg.

Increased hematocrit: 55% in men and 50% in women.

The neutrophil alkaline phosphatase score increased> 100 (no fever and infection).

3. Arterial blood oxygen saturation

Normal (92%), serum vitamin B ₁₂ increased (> 666pmol / L).

Diagnosis of polycythemia vera

1. Clinical manifestations of polyemia.

2. Hemoglobin measurement and red blood cell count increased significantly.

3. Increased hematocrit: male 0.54; female 0.50.

4. No white blood cell count> 11.0 × 10 ⁹ without infection and other reasons / L.

5. The platelet count is more than 300 × 10 ¹² / L multiple times.

6. The bone marrow showed hyperplasia, and granulocyte, red, and megakaryocytes all proliferated, especially the red blood cells.

7. Excluding secondary erythrocytosis, especially those due to cardiopulmonary insufficiency, kidney, liver, cerebellum and gynecological tumors.

Polycythemia vera treatment

The purpose of treatment is to make the blood volume and red blood cell volume close to normal as soon as possible, and inhibit the hematopoietic function of the bone marrow, thereby alleviating the disease and reducing complications.

Venous bleeding

Can reduce blood volume to normal within a short time, reduce symptoms, reduce bleeding and thrombosis. Bleeding 200 to 400 ml every 2 to 3 days until the number of red blood cells is below 6.0 × 10 ¹² / L, and the hematocrit is less than 50%. One bloodletting can maintain the effect for more than 1 month. This method is simple and can be used first. Younger patients, who have no thrombotic complications, can be treated with blood alone. However, after bloodletting, red blood cells and platelets may cause an increase in rebound, and repeated bloodletting has a tendency to aggravate iron deficiency, and attention should be paid. For elderly and patients with cardiovascular disease, bloodletting should be cautious. It should not exceed 200 ~ 300ml at one time, and the interval can be slightly extended. A large number of red blood cells can be collected by blood cell separation, but the same volume of blood as the same volume should be added, and blood should be replenished intravenously at the same time to dilute the blood.

2. chemotherapy

(1) Hydroxyurea is a type of ribonucleic acid reductase, which has a good inhibitory effect on polycythemia vera and does not cause side effects in leukemia. If the number of white blood cells is maintained at 3.5 to 5 × 10 ⁹ / L, hydroxyurea can be applied intermittently for a long time.

(2) Effectiveness of alkylating agent : 80% ~ 85%. Cyclophosphamide and L-anilide mustard (Malfalun) have a faster effect, while the remission period is busulfan and phenylbutyrate mustard. The effect can last for about six months. Nitrobacter phenylbutyrate has fewer side effects and is less likely to cause thrombocytopenia, which is its advantage. Alkylating agents are also at risk of causing leukemia, but they are less common than radionuclides.

(3) Triharvestine Domestically reported application This product is added to a 10% glucose solution by intravenous infusion once a day, continuously or intermittently until the hematocrit and hemoglobin fall to normal. The average time to remission was 60 days, with a median remission period of more than 18 months.

3. Alpha interferon treatment

Interferon has the effect of inhibiting cell proliferation, and has been used in the treatment of this disease in recent years. After 3 months of subcutaneous injection, the spleen shrinks and the number of bleeding is reduced. Remission rate can reach 80%.

4. Radionuclide therapy

32P beta rays can inhibit nuclear division and reduce the number of cells. After about 6 weeks, the number of red blood cells began to decrease, and it was close to normal in 3 to 4 months. The symptoms were alleviated. About 75% to 80% of patients were effective. If the condition does not resolve after 3 months, another dose can be given. Remission time is 2 to 3 years. 32P is at risk of turning patients into leukemia, so it has been rarely used in recent years.

5. Symptomatic treatment

(1) Secondary gouty arthritis Take allopurinol, indomethacin treatment.

(2) Itching Oral cyproheptadine, zismin, or cimetidine.

(3) Ischemic manifestations For those with extremities or cerebral ischemic manifestations, antiplatelet aggregation drugs such as aspirin and pansentine can be applied for a short period of time.