basophilic leukemia
Introduction
Introduction to basophilic leukemia Basophilicleukemia (BL), first reported by Goachim et al in 1906, with basophils as the main component in blood and bone marrow. In addition to the clinical manifestations of acute leukemia, patients also have high histamine. Urtic rash caused by blood, skin flushing and numerous gastrointestinal symptoms. There are two types of BL: one is acute in the onset and the other is basophilic granulocyte disease in the late stage of chronic CML. Some patients with acute granulocyte, monocytic leukemia have t(6;9)(p23;q34) abnormalities, basophils in the bone marrow, but not in the peripheral blood, but C(s) patients have t(9;22 (q34; q11) abnormalities, both diseases have abnormal chromosome 9 (q34), and there are increased basophils, which indicates that chromosome 9 breakpoint genes may affect basophils Generation, Peterson et al. performed ultrastructural analysis of 455 leukemia blasts in patients with acute leukemia, and found 8 cases of acute basophilic leukemia, accounting for less than 2% of acute leukemia, but Quattrin statistics from 1959 to 1973 in Naples, Italy Among the 2152 cases of hematological malignancies, the disease accounted for 4.5% of acute leukemia. The disease is immature basophils in the blood (more than 1/3 of the total number of white blood cells) and persists in the disease. process. basic knowledge The proportion of illness: more common in older men, the incidence rate is about 0.05% Susceptible people: no special people Mode of infection: non-infectious Complications: shock, cerebral hemorrhage, gastric ulcer perforation
Cause
Etiology of basophilic leukemia
(1) Causes of the disease
The cause is unknown. Most patients with basophilic leukemia are from the chronic phase of chronic myeloid leukemia (CML), but acute basophilic leukemia can also occur, but does not contain the Philadelphia chromosome.
(two) pathogenesis
Some patients with acute granulocyte, monocytic leukemia have t(6;9)(p23;q34) abnormalities, basophils in the bone marrow, but not in the peripheral blood, but C(s) patients have t(9;22 (q34; q11) abnormalities, both diseases have abnormal chromosome 9 (q34), and there are increased basophils, which indicates that chromosome 9 breakpoint genes may affect basophils Generation, Peterson et al. performed ultrastructural analysis of 455 leukemia blasts in patients with acute leukemia, and found 8 cases of acute basophilic leukemia, accounting for less than 2% of acute leukemia, but Quattrin statistics from 1959 to 1973 in Naples, Italy Among the 2152 cases of hematological malignancies, the disease accounted for 4.5% of acute leukemia. The disease is immature basophils in the blood (more than 1/3 of the total number of white blood cells) and persists in the disease. process.
Prevention
Basophilic leukemia prevention
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Basophilic leukemia complications Complications, shock, cerebral hemorrhage, gastric ulcer, perforation
1. In the treatment, a large number of basophils dissolve, releasing a large amount of histamine and causing shock is the most common clinical complication.
2. Combined stomach, duodenal ulcer, even bleeding, perforation.
3. Cerebral hemorrhage is a common complication and cause of death.
Symptom
Symptoms of basophilic leukemia Common symptoms: night sweats, eosinophilia, fever, diarrhea, bone marrow, left shift
Clinically divided into two types.
1. Acute type of disease can be seen at any age, this type is similar to acute myeloid leukemia, the onset is more rapid, clinically severe anemia, fever, fatigue, weakness, general malaise, night sweats, sore throat, cough, diarrhea, bleeding, etc. Shorter, often dying from cerebral hemorrhage, and occasionally accompanied by the release of particulate matter (especially histamine) in basophils, leading to flushing, headache, itching, hypotension or severe duodenum ulcer.
2. The chronic type has a Philadelphia chromosome, and the early blood picture, the bone marrow is similar to chronic myeloid leukemia, and the late stage resembles a slow-granular blast, so some people think that it is not an independent subtype.
Examine
Examination of basophilic leukemia
1. The white blood cell count of blood is generally (2.8 ~ 144) × 109 / L, and occasionally up to 500 × 109 / L, basophils can increase in all stages, generally 20% ~ 100%; hemoglobin and thrombocytopenia.
2. In addition to the increase of myeloblasts in the bone marrow, a large number of basophils are observed, generally accounting for 33% to 99%. The basophils are stained with toluidine blue or flash blue, which has strong positive reaction and certain specificity. Sexually, Auer bodies are rare in leukemia cells; normal basophils are negative in chloroacetate staining, but this disease can be positive, but can also be negative, myeloperoxidase and Sudan black B negative Or positive, the periodic acid Schiff reaction is negative, also positive, terminal deoxynucleotidyl transferase (TdT) positive or negative.
Under light microscopy, the degree of maturity of the original cells varies greatly from patient to patient. The nuclear shape of the original cells ranges from circular or elliptical to lobulated or deep depression. The nucleolus is often prominent, and the cytoplasm is moderate to moderate. , and have a slight basophilic, some primitive cell cytoplasm contains vacuoles, mature basophils are rare or absent, sometimes mild neutrophils and mild eosinophilia, erythroid and megakaryocytes normal.
Under electron microscopy, primordial cells often have a lymphocyte-like appearance with round, concave or irregular nuclei, medium-density chromatin, distinct nucleoli, high nuclear/cytoplasmic ratio, and mitochondria in the cytoplasm. Rare rough endoplasmic reticulum, Golgi and a large number of glycogen aggregates, the original cells contain three kinds of particles: 1 immature basophilic particles, which provide basophilic differentiation of the original cells, often showing peroxidase-positive. 2 particles may be associated with early basophilic differentiation, and peroxidase is often negative. 3 immature mast cell granules provide evidence of mast cell differentiation. Rare primordial cells have both basophilic and mast cell differentiation characteristics, and peroxidase is often negative.
3. Immunophenotype of leukemia cells The immunophenotype of leukemia cells in most patients is consistent with myeloid differentiation.
4. The cytogenetic karyotype is often inconsistent, often t(9;22), the trisomy of chromosome 21, occasionally the deletion of long arm of chromosome 7, no t(6;9) and short arm abnormality of chromosome 12. , can also be normal karyotype.
According to the clinical manifestations, symptoms and signs can be selected X-ray, CT, MRI, B-ultrasound, electrocardiogram and other examinations.
Diagnosis
Diagnosis and diagnosis of basophilic leukemia
Diagnostic criteria
The domestic diagnostic criteria are as follows:
1. Clinically, there is leukemia.
2. The basophils in the peripheral blood increased significantly.
3. A large number of basophils can be seen in the bone marrow, the original cells are >5%, the alkaloids are young, the late granulocytes are also increased, there is a nuclear left shift phenomenon, and the cytoplasm has coarse basophilic particles.
4. Organs have basophilic infiltration.
5. Absence of basophilia due to other causes.
Diagnostic evaluation: Chronic BL is the end-stage manifestation of CML. It is easier to diagnose according to the existing CML course and the accompanying blood and bone marrow of basophilia, but the treatment is very difficult.
Acute type, that is, onset of blood and bone marrow basophils, must fully rule out eosinophilia caused by other diseases, the diagnosis is difficult, because the acute type of BL is very rare, the diagnosis should be very careful .
The morphological recognition of basophils is usually not difficult, and a few atypical cases are determined by toluidine blue staining.
Differential diagnosis
1. Myeloproliferative diseases CML is the most common MPD. In most patients with CML, the peripheral blood basophils gradually increase in the course of the disease, and the bone marrow also increases accordingly. Therefore, it is necessary to identify with BL and the number of basophils. Is the key to identification, such as blood and bone marrow are >30%, should consider CML has been basophilic, that is, has been converted to BL, and <30% is still CML with basophilia, other MPD, such as polycythemia vera, idiopathic myelofibrosis may be associated with basophilic granulocytosis during the course of the disease, but the process of increase is limited, mostly below 10%, and there are clinical and experimental studies of true red and medullary fibers, respectively. Room features are often not easily confused with BL.
2. Mast cell disease This group of diseases including pigmentary urticaria, systemic mastocytosis and mast cell leukemia, due to high histamine, there are similar clinical manifestations, the identification points for histology, hematology Upper mast cell disease is involved by mast cells, while BL is involved in basophils. The morphology of the two is completely different. The difficult cases are cytochemically stained, and the mast cell chloroacetate and tartaric acid phosphatase staining are positive. The basophils are negative.
In a few cases, BL needs to be differentiated from various heavy metal poisoning and malignant tumors with increased basophilic cytoplasm. The presence and detection of primary disease is usually easy to distinguish.
