True erythropoietic disorder is a myeloproliferative disease with a predominantly erythropoietic lineage. In addition to an increase in red blood cells, hemoglobin, leukocytes and platelets, there is also an increase in red blood cell volume and blood viscosity. Skin redness, hepatosplenomegaly, and vascular and neurological symptoms are the main clinical features. Individual cases may evolve into acute leukemia in later stages, with complications seen in hemorrhage, thrombosis, and embolism, which are often the main cause of death.
Clinical manifestations.
1.General symptoms
1.1 Onset: The onset of the disease is insidious, often starting with headache, dizziness, weakness, tinnitus, etc. Some patients are occasionally detected during physical examination because they have no specific symptoms.
1.2 General manifestations: dark red skin and mucous membranes, cyanosis of nose, lips, ears, cheeks and extremities, conjunctival congestion, drunken appearance, itchy skin, and eczema, etc.
2.Specific symptoms
2.1 Neuropsychiatric system: headache is the most common, in addition, there may be dizziness, tinnitus, insomnia, syncope, paralysis, myoclonus, chorea, grand mal seizure, visual disturbance, transient blindness, diplopia, ocular muscle paralysis, etc. Psychiatric symptoms may include depression, hallucinations, amnesia, etc.
2.2 Embolism and thrombosis: It is more common in the extremities, brain, mesenteric and coronary arteries, producing paralysis, acute abdominal pain, myocardial infarction, etc. In addition, it can also cause venous and portal vein thrombosis in the lung, spleen, liver, etc.
2.3 Bleeding and anemia: Gingival bleeding is common, as well as epistaxis, skin bleeding spots and bruises. Anemia may occur in late stages.
2.4 Peptic ulcer: The incidence of peptic ulcer is significantly higher, and combined bleeding is common.
2.5 Hepatosplenomegaly: About half of the patients have hepatomegaly, and some have combined cirrhosis. The spleen accounts for about 75% to 90% of the cases, and is moderately or severely enlarged and hard.
2.6 Gout: Gout or uric acid stones are seen in a minority of patients.
Status of true red western medical treatment.
The length of survival of the disease is related to many factors such as age, disease stage and the presence of complications, especially closely related to the treatment method. Commonly used methods: intravenous bloodletting, radionuclide 32P, myelosuppressive drugs, interferon, anagrelide and symptomatic treatment. Each of these methods has advantages and disadvantages.
1.1 Intravenous bloodletting is simple and rapid, but it cannot inhibit myeloproliferation, nor can it relieve stubborn skin itching and gout attacks. Elderly people and people with cardiovascular or cerebrovascular diseases or history of thrombosis should be cautious with bloodletting.
1.2 Radionuclide 32P inhibits hematopoiesis by releasing p-rays that directly prevent nuclear division of bone marrow hematopoietic cells. The remission rate of treatment is high and the efficacy can last for six months to several years. The disadvantage is that if the dose is not properly controlled, too much can inhibit myelopoiesis, followed by an increased incidence of acute leukemia and non-hematopoietic system tumors after treatment.
1.3 Myelosuppressive drugs can be used hydroxyurea, leucovorin, benzodiazepine, cyclophosphamide, trichothecene, etc. The effectiveness rate is 80%-85%, which is suitable for those who have significant increase in blood cells, skin pruritus, gout, kidney stones, etc. who are not effective by other treatments. The disadvantage is that bone marrow suppression and distant acute leukemia can occur.
1.4 Interferon: It can improve clinical manifestations and reduce myelofibrosis, and the chemotherapy dose can be reduced accordingly. The disadvantage is that the effect is slow and should be used after other treatments are applied and the blood picture improves significantly, and the course of treatment is long, the side effects are large and the cost is relatively high.
1.5 Symptomatic treatment: allopurinol and alkaline drugs should be taken orally for hyperuricemia, and more water should be consumed for diuresis. For pruritus, use cimetidine, cyproheptadine, etc. Erythematous limb pain can be treated with low-dose aspirin and anagrelide for patients with true redness with thrombocytosis that cannot be controlled with hydroxyurea.
1.6 Targeted therapy: Since 2005, when the JAK2V617F mutation was reported to occur in chronic myeloproliferative diseases, this finding has changed the classification and diagnosis of MPD, and in the revised WHO classification system in 2008, the presence or absence of JAK2 mutation became the main diagnostic indicator of MPD. Studies have proven that JAK2 mutations exist in almost all patients with PV, and this discovery has provided a new target for the treatment of true redness, and highly effective and low toxicity targeted therapeutic drugs may become the first choice for the treatment of true redness in the near future, but these drugs are still in the laboratory or clinical trial stage, and the real application of clinical treatment is still waiting for time and expensive. The ancestral medicine, through discriminative analysis, has increasingly shown its unique advantages in the treatment of true redness with its obvious improvement of symptoms and low side effects.