pediatric erythroleukemia
Introduction
Introduction to pediatric erythroleroma Erythremia (erythroleukemia) is DiGuglielmo syndrome, also known as acute red blood cell bone marrow disease, acute non-leukemia leukemia, polycythemia, red blood marrow myeloprolifeenosis, etc., this disease is an acute or chronic bone marrow malignant proliferative disease. basic knowledge The proportion of illness: 0.031% Susceptible people: children Mode of infection: non-infectious Complications: acute leukemia fun enteritis thrush sepsis
Cause
Pediatric erythroleukemia
Causes:
The cause of the disease has not been known so far. Some people think that it is a subtype of acute myeloid leukemia, which is characterized by red and white (mainly granule) two lines of malignant hyperplasia, and finally can develop into a typical acute myeloid leukemia, the development process is: Red blood disease erythroleukemia leukemia, but not every case has such a transformation process, and some cases may die before the transformation, thus failing to show the whole process of disease development, although caused by Friend virus Erythemia is very similar to this disease, but the related virus that has caused human disease has not been isolated.
Pathogenesis:
The true erythroid clonal hematopoietic stem cell disease, the pathogenesis of which is still unclear, the excessive red blood cell production in the blood rather than the long life of red blood cells, the method of producing heme by using 59 iron and 15 nitrogen as the labeled phenylglycine to prove the patient's Erythrocyte production is more common than normal people. The lifespan of red blood cells is basically normal with 51 chrome or fluoro 32 diisopropyl phosphate. The study of the relationship between EPO and polycythemia vera shows that the EPO content in plasma and urine is not high in most patients. Increase, but significantly reduced or even not found, this is exactly the opposite of secondary polycythemia. Recent in vitro cell culture studies have shown that there may be two groups of erythroid hematopoietic stem cells in true red bone marrow, a group of abnormal cell lines The proliferation rate is faster than normal, even if it lacks EPO, and the other group is a normal cell line. Its differentiation and proliferation requires EPO. When abnormal cell lines produce a large number of red blood cells, the secretion of EPO is inhibited, and the normal cell line is relatively restless. status.
In vitro culture showed that the bone marrow of true red patients can culture red blood cell colonies without adding EPO. There are two explanations for this situation. One is considered to be true red and is highly sensitive to EPO. The other explanation is that this colony is not dependent. EPO, some people measured the EPO receptor (EpoR) on the surface of red blood cells, suggesting that the incidence of true red is not caused by the change in the number and affinity of EpoR. The incidence of true red may not be in the EpoR process, and another study on RT-SSEP and DNA. -SSEP analysis, respectively, EpoR-DNA and EpoR initiation small region analysis, no point mutations were found, and the entire PCR product encoding the EPO gene region was sequenced, and no abnormality was found, which further confirmed that the EpoR gene of true red was not abnormal.
Prevention
Pediatric erythroleukemia prevention
Because the etiology and pathogenesis are still unclear, refer to the following measures:
1. Avoid contact with harmful factors Pregnant women and children should avoid exposure to harmful chemicals, ionizing radiation and other factors that cause leukemia. When contacting poisons or radioactive materials, various protective measures should be strengthened; avoid environmental pollution, especially indoor environmental pollution; Rational use of drugs, caution with cytotoxic drugs.
2. Vigorously carry out prevention and treatment of various infectious diseases, especially viral infectious diseases, and do a good job of vaccination.
3, do a good job of eugenics, to prevent certain congenital diseases, such as 21-three body, Fanconi anemia, etc., strengthen physical exercise, pay attention to food hygiene, maintain a comfortable mood, work and rest, enhance the body's resistance.
Complication
Pediatric erythroleukemia complications Complications Acute leukemia fungal enteritis thrush sepsis
Anemia, palpitations, shortness of breath, bleeding, easy to cause infection, spleen often swollen, jaundice, can be complicated by embolism, hemiplegia, hyperuricemia, etc., can also be complicated by DIC, uric acid nephropathy.
1, anemia and hemorrhagic anemia progressive exacerbation, can occur palpitations, tinnitus, can occur varying degrees of bleeding, subcutaneous hematoma and bleeding of various organs, retinal hemorrhage, causing vision loss; digestive tract and urinary tract bleeding; intracranial hemorrhage Increased internal pressure, manifested as headache, vomiting, convulsions and coma, etc., digestive tract and intracranial hemorrhage can cause death of children, hemolysis can occur, jaundice can occur, and DIC can be complicated.
2, infection often complicated by infection, easy to spread to sepsis; common infection sites are respiratory system, skin bloated, intestinal inflammation, perianal inflammation, etc., can occur thrush, perianal fungal disease, fungal enteritis and deep fungal infections Wait.
3, leukemia cells infiltrating splenomegaly, accidental skin infiltration.
Finally developed into acute leukemia.
Symptom
Symptoms of erythro- leukemia in children Common symptoms Weak jaundice skin infiltration gas short face pale pale dizziness fever heart splenomegaly tinnitus
The clinical manifestations of this disease are similar to acute leukemia, the onset is more urgent, the course of disease is short (weeks or months), anemia is often the first symptom, progressive progressive, pale, fatigue, palpitations, shortness of breath, dizziness, tinnitus, etc. Bleeding symptoms may occur, but generally not as severe as other types, but also may cause fever, prone to infection, spleen often swollen, liver and superficial lymph nodes are rarely swollen, skin infiltration occurs by chance, may have jaundice (caused by hemolysis) Typical cases can pass the three stages of red blood disease, erythroleukemia and acute leukemia. Finally, acute myeloid leukemia is most common when it develops into acute leukemia, and a few develop acute monocytic leukemia.
The onset of sudden onset is progressive anemia, the clinical manifestations are similar to acute leukemia, bone marrow examination can confirm the diagnosis. The bone marrow image only had red system hyperplasia in the early stage, and then evolved into red system and granulosa system hyperplasia, and finally completely changed into granulosa system hyperplasia.
Examine
Pediatric erythroleukemia examination
1, blood
The blood picture may have anemia in the early stage, and there are young red blood cells in the blood film. When the red blood leukemia develops, in addition to the young red blood cells, the young cells may be seen in the blood, and the Auer body may appear in some of the young cells.
2, bone marrow
In the bone marrow of acute red blood disease, the erythroid nucleated cells in the classification of bone marrow cells are generally more than 50%, most of which are primitive, early red blood cells: and often have morphological abnormalities, such as juvenile red blood cells, giant nucleus, nuclear fragmentation , multinuclear, giant nucleus, etc., such as the classification of the young red blood cells >10%, although the erythroid nucleated cells account for only 30% of the bone marrow cells, also have diagnostic significance, in the case of erythroleukemia, the bone marrow and the young Granulocytes are often above 10%.
3. Characteristics of histochemistry
It is the PAS reaction of young red blood cells, which is often strongly positive.
Perform chest X-ray and B-ultrasound, and perform CT and angiography if necessary.
Diagnosis
Diagnosis and diagnosis of pediatric erythroleukemia
The onset of acute onset is progressive anemia. The clinical manifestations are similar to those of acute leukemia. The bone marrow examination can confirm the diagnosis. The bone marrow image only has red system hyperplasia in the early stage, and then evolves into red system and granulocyte hyperplasia, and finally becomes granular system hyperplasia. .
Differential diagnosis
In the diagnosis should be associated with erythroid leukemia, megaloblastic anemia, hemolytic anemia, myelofibrosis and other identification.
1, leukemia-like reaction
There are clinical manifestations of the primary disease (tumor, hemolytic crisis and massive blood loss); if the cause can be removed, the leukemia-like reaction can disappear quickly, in addition, the number of naive cells in the blood is not high, no thrombocytopenia, and the marrow Although the middle granules are obviously proliferating, there is no crater phenomenon, and there is no Auer corpuscle, but there are serious toxic changes, which are helpful for identification.
2. Identification of megaloblastic anemia and erythroleukemia.
3. Hemolytic anemia
Young red blood cells can be seen in the blood, and the proliferation of erythroids in the marrow can be significant, but the specific examination and treatment response of hemolysis can often help identify.
4, myelofibrosis
There is obvious swelling of the spleen, except for the young red in the blood, and the tear-shaped red blood cells can be seen outside the young cells. The bone marrow biopsy can confirm the diagnosis to help identify.
