Introduction:
Acute leukemia: Acute onset, bone marrow and peripheral blood are mainly primordial cells, and if not treated, patients often die within six months. According to the type of leukemia cells, it is clinically divided into acute lymphoblastic leukemia (ALL) and acute non-lymphocytic leukemia (ANLL), each of which has several types. At present, the common classifications at home and abroad are as follows: 1ANLL is divided into 7 types, namely granulocyte leukemia undifferentiated (M1), granulocyte leukemia partial differentiation (M2), promyelocytic (M3), granulocyte-monocyte Type (M4), monocyte type (M5), erythroleukemia (M6), megakaryocyte type (M7); 2ALL is divided into L1, L2 and L3 types. In recent years, it is divided into T and B according to the immunological characteristics of cells. , pre-B, normal and undifferentiated.
Cause:
The exact cause of human leukemia is still unknown. Many factors are thought to be related to the development of leukemia. The virus may be the main factor, in addition to ionizing radiation, chemical poisons or drugs, genetic factors.
1. The viral etiology of viral human leukemia has been studied for decades, but so far only adult T-cell leukemia has been caused by viruses. Other types of leukemia have not been able to confirm their viral factors and are not contagious.
2. Ionizing radiation ionizing radiation has leukemia-like effects, and its effect is related to the size of the radiation dose and the irradiation site. A large dose or multiple doses of radiation may cause leukemia.
3. The chemical substance benzene induced leukemia is relatively positive. Benzene-induced acute leukemia lives in acute granules and erythroleukemia.
4. Genetic factors The incidence of certain leukemias is related to genetic factors.
Acute leukemia is clinically divided into acute myeloblastic leukemia (AML) and acute lymphoblastic leukemia (ALL).
symptom:
[clinical manifestations]
1. The onset of leukemia is rapid or slow, and children and adolescents have a rapid onset of illness. Common first symptoms include fever, progressive anemia, significant bleeding tendency, or bone and joint pain. The patients with slow onset are mostly elderly and some young patients, and their condition is gradually progressing. Most of these patients have progressive fatigue, weakness, pale face, shortness of breath after fatigue, loss of appetite, weight loss or fever of unknown origin are the first symptoms. In addition, a small number of patients may have convulsions, blindness, toothache, swelling of the gums, pericardial effusion, and paraplegia of both lower extremities as the first symptom onset.
2. Fever and infection
A. Fever is one of the most common symptoms of leukemia, and can occur at different stages of the disease and have varying degrees of fever and heat. The main cause of fever is infection, among which angina, stomatitis and perianal inflammation are the most common, pneumonia, tonsillitis, gingivitis, perianal abscess are also common. Ear inflammation, enteritis, phlegm, pyelonephritis, etc. can also be seen, severe infection can also occur sepsis, sepsis and so on.
B. Infected pathogens are more common in bacteria, and in the early stage of the disease, Gram-positive cocci are the main. Although viral infections are rare but often more dangerous, cytomegalovirus, measles or varicella virus infections may be complicated by pneumonia.
3. Hemorrhagic hemorrhage is also a common symptom of leukemia. The bleeding site can be spread throughout the body. Skin, gums and nasal bleeding are the most common. There may also be retinal, intra-earal hemorrhage and visceral hemorrhage such as intracranial, digestive tract and respiratory tract. Women's menorrhagia is also more common and may be the first symptom. AML M3 and M5 subtypes are more severely bleeding, especially in patients with M3 who are prone to concurrent disseminated intravascular coagulation (DIC) and intracranial hemorrhage.
4. Anemia can occur early, and in a few cases, refractory anemia can occur several months or years before diagnosis, and later develop into leukemia. Patients are often accompanied by symptoms such as fatigue, pale complexion, palpitations, shortness of breath, and swelling of the lower extremities. Anemia can be seen in all types of leukemia, but more common in elderly AML patients, many patients often with anemia as the first symptom.
5. Leukemia cell infiltration signs
A. Liver, splenomegaly, swollen lymph nodes
B. Nervous system: the main lesion is hemorrhage and leukemia infiltration
C. Bone and joint: Bone and joint pain is one of the important symptoms of leukemia, and ALL is more common.
D. skin; there may be two types of specific and non-specific skin lesions, the former manifested as maculopapular rash, pustules, masses, nodules, erythroderma, exfoliative dermatitis, etc., more common in adult monocytic leukemia, the latter More manifestations of skin ecchymosis, spots and so on.
E. Oral: Gingival swelling, hemorrhage, leukemia infiltration is more common in AML-M5. In severe cases, the entire gum can be extremely hyperplasia, swelling such as sponge-like, surface ulceration and bleeding.
F. Heart: Most manifestations are myocardial leukemia infiltration, hemorrhage and epicardial hemorrhage, pericardial effusion.
G. Kidney: Up to 40% of leukemia patients with kidney disease.
H. Gastrointestinal system: manifested as nausea and vomiting, loss of appetite, bloating, diarrhea, etc.
I. Lung and pleura: mainly infiltrating the alveolar wall and lung space, but also infiltrating the bronchus, pleura, blood vessel wall and so on.
J. Others: The uterus, ovaries, testes, prostate, etc. can all be infiltrated by white blood cells. Female patients often have vaginal bleeding and menstrual cycle disorders. Male patients may have decreased libido.
【Diagnostic criteria】
1. The clinical symptoms are sudden and high fever, progressive anemia or significant bleeding, and the body is sore.
2. Signs of skin bleeding spots, sternal tenderness, lymph nodes, hepatosplenomegaly.
3. Laboratory:
A. Blood-like white blood cells are always significantly increased (or reduced), and primitive or immature cells may appear.
B. Bone marrow, bone marrow nucleated red blood cells account for less than 50% of all nucleated cells, primordial cells ≥ 30%, can be diagnosed as acute leukemia; such as bone marrow nucleated red blood cells ≥ 50%, primordial cells account for ≥ 30% of non-erythroid cells Can be diagnosed as acute erythroleukemia.
diagnosis:
C. malignant histiocytosis;
D. Idiopathic thrombocytopenic purpura.
complication:
1. Infection: due to leukemia caused by normal leukopenia, especially neutropenia, and chemotherapy and other factors also lead to the lack of granulocytes, making patients prone to serious infection or sepsis. Common bacteria causing infection are: Gram-positive bacteria, such as Staphylococcus aureus, hemolytic streptococcus, coryneform bacteria and other Gram-negative bacilli, such as Pseudomonas aeruginosa, Escherichia coli, Klebsiella. The mold infection is caused by Candida albicans, Aspergillus, Mucor, and the above-mentioned fungal infections occur in patients with long-term granulocytosis or persistent fever and antibiotics are not sensitive. Some patients receiving corticosteroid treatment are more susceptible to viral infections due to low cellular immune function, such as varicella zoster virus and herpes simplex virus. In addition, Pneumocystis carinii infection is also common, and upper respiratory tract infections and pneumonia are common types.
2. Intestinal failure: due to the treatment of leukemia chemotherapy drugs, radiotherapy means affecting gastrointestinal function, leading to fear of gastric failure, nutritional supplementation of patients has become a prominent problem, currently using subclavian vein cannula to the superior vena cava High-nutrient infusion only solves some problems, and nutritional deficiency can cause complications such as pneumonia and enteritis.
3. High uric acid blood test: normal people due to nucleic acid metabolism decomposition, daily uric acid excretion of 300 ~ 500mg. Leukemia patients can increase uric acid output by several tens of times due to nucleic acid breakdown of a large number of leukemia cells. When patients receive chemotherapy, radiotherapy and other treatments, hyperuricemia occurs, and the application of corticosteroids can increase hyperuricemia. High concentrations of uric acid quickly over-saturate and precipitate, causing extensive damage to kidneys and uric acid stones, which can lead to No urine, no urine. Therefore, leukemia patients must supplement a sufficient amount of fluid to ensure a certain amount of urine, and take allopurinol, such as renal failure, you must limit the amount of fluid replacement, and dialysis treatment.
4. Bleeding: Leukemia patients with malignant hyperplasia of leukemia cells, platelets are significantly reduced, easy to cause respiratory, digestive tract, urinary bleeding, especially intracranial hemorrhage, so it is necessary to take active hemostasis according to the cause, including infusion of concentrated platelets.
5. Pulmonary disorders: As a result of normal mature neutrophils in leukemia patients, immune function is reduced, often leading to lung infections. In addition, leukemia cells, infiltration can block small blood vessels in the lungs, bronchial dyspnea, respiratory distress syndrome, chest X-ray can have hairy glass or miliary network, can be used for experimental treatment of lung radiation.
6. Electrolyte imbalance: In the course of treatment of white backlog disease, excessive potassium is often destroyed due to excessive destruction of leukemia cells or due to chemotherapy-induced renal damage. Due to chemotherapy, the dietary taste is poor, the digestive system is dysfunctional, and the amount of hypoxia is low. Or due to the destruction of leukemia cells, the release of phosphorus increases, resulting in low calcium and the like. Therefore, attention should be paid to the electrolyte concentrations of potassium, calcium and sodium during the treatment.
7. Disseminated intravascular coagulation (DIC): The disseminated blood vessel is a group of severe hemorrhagic syndromes.
treatment:
1. Principles of treatment: The general principle of treatment is to eliminate leukemia cell populations and control the proliferation of leukemia cells, and to eliminate various clinical manifestations caused by leukemia cell infiltration.
2. Supportive treatment
(1) Pay attention to rest: When you have high fever, severe anemia or obvious bleeding, you should rest in bed. Eat high-calorie, high-protein foods to maintain water and electrolyte balance.
(2) Prevention and treatment of infection: Severe infection is the main cause of death, so prevention and treatment of infection is very important. A "sterile" ward or area should be placed in the ward to isolate people with low neutrophil counts or chemotherapy. Pay attention to the skin around the mouth, nasopharynx, and anus to prevent mucosal ulcers, erosion, and bleeding. Once it appears, it should be treated promptly. Food and utensils should be sterilized first. Oral non-absorbed antibiotics such as gentamicin, colistin and anti-fungal bacteria such as nystatin, vancomycin, etc. to kill or reduce intestinal bacteria and mold. For patients with existing infections, bacterial culture and drug susceptibility tests are performed before treatment in order to select effective antibiotic treatment. In general, fungal infections can be nystatin, clotrimazole, miconazole, etc.; virus infection can be selected Ara-c, ribavirin. When the fine particles are reduced, the white blood cells and plasma can be administered intravenously for symptomatic treatment.
(3) Correct anemia: significant anemia can be infused with red blood cells or fresh whole blood as appropriate; autoimmune anemia can be used for adrenal cortical hormone, testosterone propionate or protein anabolic hormone.
(4) Control bleeding: Chemotherapy for leukemia, so that the disease is relieved is the most effective way to correct bleeding. However, thrombolysis is prone to hemorrhage before chemotherapy is relieved, and it can be prevented by oral administration of Anluo blood. Available when there is severe bleedingAdrenocorticotropic hormone, lose whole blood or platelets. Acute leukemia (especially early granules), complicated with DIC, should be treated with heparin rapidly after diagnosis. When DIC is combined with fibrin, anti-fibrinolytic drugs (such as p-carboxybenzylamine, hemostasis) are given at the same time as heparin treatment. Acid, etc.). Fresh blood or plasma can be infused if necessary.
(5) Prevention and treatment of hyperuricemia: In patients with high white blood cell counts, when a large number of white blood cells are destroyed and decomposed, blood uric acid is increased, and sometimes the urinary tract is obstructed by uric acid stones, so special attention should be paid. Urine volume, and urine sediment and urine acid concentration determination, in addition to encouraging patients to drink more water, in addition to steroid 10mg / kg · d, three times orally, for 5 to 6 days; when blood uric acid > 59um01 / L A large amount of infusion and alkalized urine is required.
3. Chemotherapy: Chemotherapy is the main method for the treatment of acute leukemia. It can be divided into two stages: remission induction and maintenance treatment. During this period, intensive treatment, consolidation treatment and central nervous system prevention and treatment can be added. Remission induction is a strong chemotherapy combined with large doses of multiple drugs, in order to quickly kill a large number of leukemia cells, control the disease, achieve complete remission, and lay a good foundation for future treatment. The so-called complete remission refers to the symptoms of leukemia, the signs disappear completely, the blood and bone marrow are basically restored to normal. When the acute leukemia is treated, the number of leukemia cells in the body is estimated to be 5×1010～13, and the body still reaches the remission standard after treatment. There are a considerable number of leukemia cells, estimated to be below 108-109, and there may still be leukemia cell infiltration in some hidden places outside the marrow. Maintenance Therapeutic A series of small-dose, more palliative treatment regimens for extended treatment over a longer period of time with the goal of consolidating complete remission from remission induction and allowing patients to survive this long-term “disease-free” condition. Reach a cure. Consolidation treatment is after maintenance treatment. Before the maintenance treatment, the mitigation induction program was repeated in the case of the patient's permission. Intensive therapy is a recurrence of the original mitigation induction in the course of several courses of maintenance therapy. Central nervous system prophylactic treatment should be performed immediately after the induction treatment is relieved to avoid and reduce the occurrence of central nervous system leukemia. A complete treatment plan should follow the above principles.
(1) Treatment of acute lymphoblastic leukemia
1 Remission induction therapy: The commonly used chemotherapy regimen for treatment of ALL is VP regimen, based on VP regimen and DRN (daunorubicin), ADM (doxorubicin), Ara-c, L-ASP (L-asparaginase) And drugs such as 6-MP constitute many effective multi-drug combination programs. The rate of CR (complete remission) in children with initial treatment can reach 90% to 95%; adults can also reach 80% to 90%. The multi-drug regimen is mainly used for the treatment of refractory and relapsed cases.
Chemotherapy regimen
VP program VCR 2mg intravenously, once a day on the first day
PDN 60mg divided orally, day 1-7
DVP regimen DRN 1mg/kg intravenously, on the first day, once a week for 4 to 6 weeks for a course of treatment
VCR 1.5mg/m2 intravenous, first day, once a week
PDN 40mg/m2 orally, day 1-8
POMP program PDN 60mg / d divided into 5 days for a course of treatment
VCR 2mg intravenous, day 1
MTX 30mg intravenous, 2nd, 5th day
6-MP 100mg orally
VDCP program DRN 40mg/m2·d intravenous injection, the first, second, third, fifteenth, sixteenth, and seventeenth days for a course of treatment
VCR 2mg intravenous, day 1, 8, 15, 21
CTX 0.4-0.8/m2 intravenous, day 1, 15
PDN 40-60mg/m2·d Oral, after 1-14 days, reduction
DVP+ASP regimen VCR 2mg intravenously, on the first day, once a week on the 15th day for bone marrow examination, if there are still leukemia cells reuse DRN50mg/m2, 4 weeks for a course of treatment
DRN 50mg/m2 intravenous, day 1, 2, 3
PDN 60mg/m2 orally, day 1-28
L-ASP 600u/m2 intravenous, day 17-28
Note: The VP and DVP programs are applicable to child cases.
2 maintenance treatment: After using the above program to achieve CR, should continue to use the original program to consolidate the efficacy. Those who use VP and VDP programs should continue for another 2 to 3 weeks; those who use the POMP program can use two more courses. During the remission period, 6-MP100 mg/d was used for 7 days, followed by intravenous administration of 400 mg of CTX; 5 mg of MTX was given for 7 days, intravenously or orally, on days 1, 5, and 9; the above treatment was repeated sequentially after 3 days.
3 relapse treatment: can continue to use VP regimen or Ara-C5-10mg, once a day intravenous injection, a total of 4 times, or DRNlmg / kg · d, intravenous injection, a total of 4 days.
(2) Treatment of acute non-lymphocytic leukemia
1 Remission induction: The treatment plan is shown in Table 30-3.
Chemotherapy regimen
DA program DRN 30～40mg/M2·d intravenous injection, 1st to 3rd day interval 1~2 weeks repeat
Arc-c 100～160mg/M2 divided orally, day 1-7
VPP regimen VCR 2mg intravenously, repeated treatment on the first day interval 1-2 weeks
Ara-C 100～150mg intravenous, day 1 and 5
DRN 30mg intravenously, days 1 and 2
COAP regimen CTX 200mg intravenously, repeated treatment every other week on days 1, 3, and 5.
VCR 2mg intravenous, day 1
Ara-c 100～150mg intravenous drip for 4 days
PDN 200mg/d divided orally
HOP regimen VCR 2mg intravenously, repeating on day 1 interval 1-2 weeks
HRT 2～4mg intravenous drip, 3 hours drip; 2nd, 7th day
PDN 40mg/d divided orally, day 1-8
Note: VPP program can replace DRN with ADM, the dose is 20mg, intravenous injection, day 1, 2
2 maintenance treatment: generally with MTXl5mg intramuscular or oral, 6-MPl00mg / d, CTX 200mg / m2 orally, once a week, long-term maintenance, and after the maintenance treatment began l / 2, 1, 2, 4, 7, 16 months plus the original induction program to consolidate and strengthen, once every six months after 16 months, at least 2 to 4 years.
prevention:
First, prevention
1. All workers who are exposed to ionizing radiation and toxic chemical substances such as benzene and their derivatives at work should strengthen protective measures, carefully follow the routine of work, conduct regular physical examinations, and actively treat patients with abnormal blood.
2. There are festivals in life, there are always living quarters, avoiding the cold and heat, combining work and rest, so that the body is in a good state, the so-called: "righteousness and bones, evil can not be done."
3. People should be optimistic and uplifting, feel comfortable, and avoid anger, the so-called: "spiritual self-defense, sickness and never".
Second, care
1. Mental care: Medical staff and their families should calm the patient's mood, overcome the pessimistic disappointment of the disease, encourage patients to actively fight the disease, and cooperate well with the treatment.
2. Pay attention to the patient's rest: especially during the treatment of unstable conditions, reduce or avoid visits, and avoid activities in public places.
3. Oral and perianal cleansing care: urge patients to use daily light salt water, hydrogen peroxide, nitrofurazone gargle or scutellaria scutellariae to prevent oral infections; keep the urine and urine unobstructed, pay attention to cleanliness and prevent infection of skin and mucous membranes. You can wash the perianal with dehydrated grass and dandelion.
4. Nutritional catering: To allow patients to eat nutritious and delicious foods, eat high-vitamin foods, such as fresh vegetables, fish, eggs, large meat, turtles, etc., eat spicy products, such as onions, peppers Wait.
5. Encourage patients to drink more water, eat fresh fruit, avoid alcohol and tobacco.
6. Keep the air fresh in the room, clean and disinfect the floor, and medical staff should wear a mask.
[prognosis]
1, acute leukemia, frequent onset, if not timely and correct treatment of the disease rapidly deteriorated, especially combined infection, bleeding and more serious, is the main cause of death.
2. In recent years, due to the development of immunology and molecular medicine, the diagnosis of acute leukemia has progressed from cell morphology and histochemistry to cellular immunological markers, genetics, and molecular medicine. In the treatment, the efficacy of acute leukemia is significantly improved due to the emergence of new anti-leukemia drugs and the development of cell dynamics and the application of many effective supportive therapies and techniques. For example, in children with acute lymphoblastic leukemia, the complete remission rate is over 95%, and the three-year disease-free survival rate has exceeded 50%. It is currently believed that a considerable number of children are likely to be cured. The complete remission rate of adult acute lymphoblastic leukemia and acute myeloid leukemia (urgent non-leaching) has reached more than 60%, and the long-term disease-free survival rate is close to 30%.
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