In the latest "2023 China Thrombocythemia Blue Book" report, 904 patients participated in the survey, of which the most patients were diagnosed with essential thrombocythemia, accounting for about 85%, of which 63.3% of the patients were found to have abnormally elevated platelets during physical examination, and 21.1% of patients were found during treatment in local hospitals due to repeated dizziness. Essential thrombocythemia is a subtype of myeloproliferative neoplasm, which was considered a very rare hematologic disease in the past, but in recent years, with the improvement of the physical examination system and the advancement of diagnostic standards and techniques for this disease, it has been found that this disease is not uncommon.
Recently, Chief Physician Zhang Lei, Secretary of the Discipline Inspection Committee and Deputy Secretary of the Party Committee of the Blood Disease Hospital of the Chinese Academy of Medical Sciences (Institute of Hematology, Chinese Academy of Medical Sciences), was invited to the live broadcast room of the Media Federation of Medical Sciences to partner with Lin Xin, the gold medal host of the national health program, to discuss essential thrombocythemia and other related blood diseases with netizens.
The incidence of essential thrombocythemia is low and is common in middle-aged and older adults
First of all, Chief Physician Zhang Lei introduced to us what is essential thrombocythemia, which is a BCR-ABL-negative classical myeloproliferative neoplasm (MPN) that originates from the clonal proliferation of hematopoietic stem/progenitor cells.
The pathological manifestations are mainly an increase in the number and morphological abnormalities of megakaryocytes in the bone marrow, resulting in persistent production of excessive platelets, and some platelets are also associated with dysfunction. Because the onset of this disease is relatively slow, it is not easy to appear, it is difficult to detect in the early stage, only when there are physical examinations or other symptoms, such as headache, or numbness of the hands and feet, and then check to find thrombocytosis, and some patients have liver and spleen enlargement, and even more cerebral infarction, myocardial infarction or thrombosis and other thrombotic manifestations or bleeding manifestations such as cerebral hemorrhage, in addition, a small number of patients may progress to myelofibrosis or even acute leukemia.
This disease sounds terrible, but its incidence is relatively low, about 1~2.5/100,000 people suffer from this disease. Chief Physician Zhang Lei also told us that the incidence of this disease is mostly found in middle-aged and elderly patients, concentrated in about 50~60 years old, and young patients are not uncommon, and the incidence rate is very low compared to children.
The presence of these 4 types of symptoms should raise suspicion for essential thrombocythemia
Although the survival time of such diseases as fever (in the absence of serious complications) is no different from ordinary people, complications may also affect the quality of life of patients, so how to identify is also very important.
1. Thrombosis or embolism: It is more likely to occur in splenic vein, hepatic vein, mesenteric vein, lower limb vein, axillary artery, intracranial artery and acral artery, cerebral artery, cardiac artery, etc., and often causes corresponding symptoms, and patients present with abdominal pain, cyanosis, swelling, and necrosis of the toes. Cerebrovascular microthrombosis can lead to transient ischemic attack, and when venous thrombosis of the lower extremities breaks off, it can be complicated by fatal pulmonary embolism;
2. Bleeding: spontaneous bleeding, recurrent attacks, long intermittent period, common gastrointestinal bleeding, gingival bleeding, nosebleeds, hematuria, respiratory tract bleeding, skin and mucosal ecchymosis and other symptoms;
3. Splenomegaly: about 50%~80% of patients have splenomegaly, most of them are moderate splenomegaly, and splenomegaly is rare. About half of patients have mild hepatomegaly, usually without large lymph nodes;
4. Accompanying symptoms: about 20%~30% of patients have weight loss, night sweats, low-grade fever and itching, as well as dizziness, fatigue, fatigue, numbness of hands and feet, extremity pain and other symptoms;
Therefore, once you have uncomfortable symptoms, you must go to a regular hospital for treatment in time.
It is important to note that about 20% of patients with essential thrombocythemia, especially younger patients, are asymptomatic at the time of onset.
How is essential thrombocythemia diagnosed?
It is very prudent for specialists to wear a hat for the disease, so when determining whether it is ET, the World Health Organization (WHO) 2022 diagnostic criteria for essential thrombocythemia will be consulted.
Essential thrombocythemia is diagnosed if the 4 main criteria or the first 3 major criteria plus the minor criteria are met. Main Criteria:
1.血小板计数≥450×109/L；
2. Bone marrow biopsy showed that megakaryocytes were highly proliferated, and the number of mature megakaryocytes with large cell bodies and over-lobulated nuclei increased, without significant hyperplasia or left shift of granulogranules and erythroid lines, and reticular fibers rarely increased (grade 1);
3. Does not meet the WHO diagnostic criteria for BCR-ABL+ chronic myeloid leukemia, polycythemia vera, primary myelofibrosis, myelodysplastic syndrome or other myeloid tumors;
4.有JAK2、CALR或MPL基因突变。
Secondary criteria: presence of clonal landmarks or evidence of non-responsive thrombocytosis.
Hearing this, netizens in the live broadcast room asked: "Is it normal for us to take blood to diagnose?" Chief Physician Zhang Lei replied: "No, bone marrow biopsy and bone marrow genetic examination can be confirmed at the same time as blood drawing." If you want to know the diagnosis, you need to know what are the risk factors for ET: mainly age, whether there is a history of blood clots, gene mutations, whether there are cardiovascular risk factors such as hypertension and diabetes. Better treatment is possible only by grouping by class.
Patients with essential thrombocythemia should pay attention to these 5 points in their daily life
So in the process of treatment, what should ET patients pay attention to? Finally, Chief Physician Zhang Lei also instructed:
1. Dietary care: Don't eat anything too greasy. When eating, we should pay attention to a reasonable combination of high-nutrient food, eat more fresh fruits, high-lipoprotein, especially do not smoke, limit alcohol consumption, and do not have alcoholism;
2. Rest guidance: go to bed early and get up early, maintain good work and rest habits, exercise appropriately, and combine work and rest;
3. Psychological counseling: maintain an optimistic attitude, avoid anxiety and fear, and family members should take the initiative to communicate with the patient, comfort the patient, help the patient face the reality correctly, establish confidence in overcoming the disease, and actively cooperate with the treatment. Convey positive emotions among patients, exchange information about disease improvement, encourage each other, and promote recovery;
4. Daily life: avoid bumps and bruises, pay attention to keeping warm, and avoid causing wind chill and cold. Keep the mouth clean and clean, rinse the mouth with mouthwash before and after eating, and use a soft brush when brushing teeth to prevent damage to the oral mucosa, which can cause secondary bleeding;
5. Medication nursing: follow the doctor's instructions, communicate with the doctor at any time, adjust the medication under the guidance of the doctor, and do not reduce or stop the drug by yourself;
At the same time, we also hope that every patient can score the symptoms regularly, the purpose of which is to be able to observe the development of the disease, so that the doctor can adjust the treatment plan at any time.
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