eosinophilic leukemia
Introduction
Introduction to eosinophilic leukemia Eosinophilic leukocytic leukemia (EL) is a rare form of leukemia characterized by peripheral eosinophilia in peripheral blood and bone marrow, often involving the heart, lungs, and nervous system, with progressive anemia and thrombocytopenia. basic knowledge Sickness ratio: 0.0001% Susceptible people: no special people Mode of infection: non-infectious Complications: anemia, epilepsy
Cause
Eosinophilic leukemia
(1) Causes of the disease
A small number of patients can be transformed from idiopathic hypereosinophilic syndrome, or it can be a late manifestation of very few acute lymphoblastic leukemia or chronic myeloid leukemia.
(two) pathogenesis
The pathogenesis has not been elucidated. It has been reported that hematopoietic cells overexpress the Wilms tumor gene, which leads to inhibition of apoptosis. Patients may have clonal chromosomal abnormalities but no marker chromosomes.
Prevention
Eosinophilic leukemia prevention
Active treatment of the primary disease.
Complication
Eosinophilic leukemia complications Complications, anemia, epilepsy
1. When anemia is severe, it can be complicated by anemia of heart disease.
2. Combined infection with fever, mainly seen in pulmonary infections.
3. Concurrent neurological symptoms, mental disorders, also obscure and hemiplegia, epilepsy.
Symptom
Symptoms of eosinophilic leukemia Common symptoms Lymph node enlargement Eosinophils increase mental disorders, underarm axillary lymphoma, dyspnea papules
The length and speed of the disease are related to the degree of eosinophil maturation, so it is generally divided into two types in clinical practice.
Acute type
The disease is different from general leukemia in that it has less bleeding, mainly eosinophil infiltration of various organs, leading to dysfunction. In addition to the involvement of liver, spleen and lymph nodes, it also manifests as heart, lung and central nervous system involvement. Clinical manifestations of progressive heart failure, may have galloping, pericardial friction sound; cough, difficulty breathing; if there is central nervous system infiltration, manifested as mental disorders, delusions, blurred vision, ataxia, hemiplegia, etc., in addition, The skin may have erythema, papules, nodules, and the like.
2. Chronic type
The onset is slow, the course of disease can be extended to 2 to 8 years, there is fatigue, anemia, liver and spleen and lymph nodes.
Examine
Examination of eosinophilic leukemia
1. Common anemia and thrombocytopenia in blood, the number of white blood cells is significantly increased, up to (50 ~ 200) × 109 / L, eosinophils in blood tablets accounted for 20% to 90%, most of them in more than 60%, of which eosinophilic The young and late granules are mainly increased, and the original granules and early granules are rare.
2. Bone marrow image In addition to the increased proportion of myeloblasts, eosinophils increased significantly and left shifted, according to cell morphology can be divided into three types:
1 granulocyte type: both blood and bone marrow have increased granulocytes.
2 immature cell type: in addition to the obvious increase of bone marrow naive eosinophils, such cells can also be seen in peripheral blood.
3 mature cell type: mainly mature eosinophils, including eosinophilic, promyelocytes increased, normal or slightly increased.
3. Chromosome examination There are often three-body types of chromosomes 8 and 10, 4q and 45X, 49XY and other chromosome abnormalities.
4. Cell culture The growth of CFU-GM in peripheral blood cells is similar to that of chronic myeloid leukemia. The growth pattern combined with chromosome examination can be used to distinguish eosinophilic leukemia from other causes of eosinophilia.
Diagnosis
Diagnosis and identification of eosinophilic leukemia
Diagnostic criteria
There is no uniform diagnostic standard in the world, and the domestic diagnostic criteria are as follows.
1. Clinical manifestations of leukemia.
2. Peripheral blood eosinophils increased significantly and continued to increase, most of them up to 60%, and often have naive eosinophils.
3. Bone marrow Eosinophilia, abnormal morphology, left shift of the nucleus, there are coarse eosinophilic granules in the immature granulocytes at each stage, and the original cells are >5%.
4. Organs have eosinophil infiltration.
5. Can exclude parasitic diseases, allergic diseases, connective tissue diseases, high eosinophilic syndrome, chronic myeloid leukemia and other causes of eosinophilia.
Diagnostic evaluation: EL is rare, the diagnosis must be very careful, and it is sufficient to exclude eosinophilia caused by other causes, hematological examination, in addition to eosinophilia, it should have abnormal morphology, naive eosinophils appear in the blood Cells, 5% of the original cells in the bone marrow are also necessary conditions. In case of suspicious cases with insufficient conditions, the primary disease should continue to be searched, and the changes in the condition should be carefully observed.
Differential diagnosis
1. Idiopathic hypereosinophilic syndrome (IHES)
IHES also has a marked increase in white blood cells and eosinophils. Eosinophils infiltrate the heart, lungs and nervous system. Anemia and/or thrombocytopenia can also occur during the course of the disease. Therefore, it is easy to be confused with EL. :
The morphology of eosinophils in 1IMES was normal, and there were no immature eosinophils in peripheral blood. The proportion of blast cells in bone marrow was within the normal range, which was different from EL.
2IHES has a relatively long course of disease and slow progress, while EL progresses rapidly, and anemia and thrombocytopenia are progressive, but some IHES clinical progress is also rapid, and short-term death can be determined mainly by blood and bone marrow.
2. Malignant disease with eosinophilia
(1) M4E0 type of acute myeloid leukemia (AML): acute myelomonocytic leukemia with eosinophilia, which has clinical manifestations of AML, increased eosinophils in blood and bone marrow, and abnormal morphology. Therefore, it needs to be identified with EL:
1M4E0 usually only slightly increased the eosinophils in blood and bone marrow <30%, and the main components are still primordial cells and primitive, naive monocytes, which is obviously different from EL.
2 difficult cases feasible chromosome and gene detection, M4E0 has a marker chromosomal abnormality, namely inv (16) (p13; q22), and the corresponding fusion gene MYH11 / CBF, EL also has chromosomal abnormalities, but are non-significant, such as 8, Chromosome 3, 4q, 45X, 49XY, etc.
(2) Acute lymphoblastic leukemia (ALL): A small number of ALL has increased eosinophils in the blood and bone marrow during the course of the disease, and the central nervous system is often involved, so it needs to be identified with EL:
1ALL usually has an acute onset, and EL mostly hides onset.
2ALL patients often have superficial lymphadenopathy, while EL patients are relatively rare.
3EL patients have common cardiac involvement and involve the whole heart (endocardium, myocardium, pericardium), while the ALL heart is usually not invaded, only the myocardial toxicity caused by anthracyclines is low, and the incidence is also low.
4 immunophenotyping, ALL expresses lymphocyte series differentiation antigen, and EL expresses myeloid cell differentiation antigen.
Before the occurrence of eosinophilia in 5ALL, most of the typical clinical and laboratory characteristics of ALL.
(3) myeloproliferative diseases (MPD): chronic myeloid leukemia (CML), polycythemia vera (PV) and idiopathic myelofibrosis (IMF) and other MPD in the course of the disease, especially in the late stage may appear eosinophils The number of cells increases, and the identification points are:
1CML, PV and IMF have a long course of disease before eosinophilia, and there are corresponding clinical manifestations and laboratory tests. CML is mainly composed of metaphase naive neutrophils, and PV is mainly red blood cells. Increased and corresponding multi-blood clinical manifestations, IMF is characterized by splenomegaly and granules, erythroblastemia.
2EL common heart, lung and nervous system lesions are often absent in MPD.
3CML can also be distinguished by the marker Ph chromosome and the specific fusion gene BCR/ABL and EL.
4IMF bone marrow biopsy showed significant fibrosis and was not difficult to identify with EL.
(4) Hodgkin's lymphoma: the course of the disease is often accompanied by eosinophilia, due to fever at the same time, lymphadenopathy, lymph node biopsy must be performed before diagnosis, so it is easier to distinguish from EL.
3. The most common eosinophilia
Clinically, parasitic infections, allergies, skin diseases, and rheumatic diseases are the most common causes of eosinophilia and are important for identification with EL.
(1) Parasitic infection: Parasite larvae migrate and invade tissues in vivo are pathological mechanisms that induce eosinophilia. Feces repeatedly search for eggs and serologically detect corresponding antibodies. Specific antigen skin tests are identified. The main means, after the corresponding deworming treatment, eosinophils down to normal is also the basis for diagnosis.
(2) allergic reactions: bronchial asthma, angioedema, drug allergy, serum disease, etc. can cause eosinophilia, according to the corresponding medical history, clinical manifestations, and EL identification should be no difficulty.
(3) skin diseases: pemphigus, herpes-like dermatitis, etc. can cause white blood cells and eosinophilia, and many other skin diseases have similar complications. Because of clinically obvious skin lesions, EL usually has no skin involvement, and identification It's easier.
(4) Rheumatic diseases: rheumatoid diseases such as nodular polyarteritis, rheumatoid arthritis, and special connective tissue diseases (such as eosinophilic fasciitis), eosinophilic muscle syndrome (by ingestion L-tryptophan-contaminated foods can cause eosinophilic elevation, and the clinical manifestations of the primary disease are characteristic, and are usually not easily confused with EL.
4. Lung disease with eosinophilia
Because the lungs are often involved in EL, it must be differentiated from various lung diseases with eosinophilia.
(1) Loeffer syndrome: characterized by pulmonary imaging findings showing migratory shadows and eosinophilia, and some patients are caused by parasite infection after larval migration to the lungs, due to their lung shadows are migratory And disappear faster, is an acute self-limiting course, are different from EL.
(2) pulmonary infiltration with eosinophils (PIE) syndrome: manifested as chronic, recurrent respiratory symptoms, imaging findings showed pulmonary pleomorphic infiltration, and blood eosinophilia, most The cause of the patient is unknown, and only a few are related to Mycobacterium tuberculosis, Brucella, Coccidioidomycosis and viral infection, and the lesion is limited to the lung is the key to identification.
(3) Tropical eosinophilia: the status of immune response after infection with filarial worms, mainly manifested as dry cough, wheezing, low fever, wheezing sounds of both lungs, imaging examination showing interstitial shadows of lung interstitial, similar to miliary Sexual tuberculosis, the disease is now rare in China, mainly in India and Southeast Asian countries, patients often have elevated serum IgE, can detect anti-filaria antibody, citrate ethylamine (Hai Qunsheng) quickly improved after treatment, both For the identification point.
5. Eosinophilic lymphogranuloma
It is a rare disease, manifested as superficial lymphadenopathy with blood eosinophilia, lymph node pathology is granuloma formation with eosinophil infiltration, the disease responds well to corticosteroids, but some patients can be repeated after stopping the drug.
6. Other diseases with eosinophilia
Rare hereditary eosinophilia is autosomal dominant, with a family history, blood eosinophils are only slightly elevated, benign, occasionally extensive caseous lymph node tuberculosis, after radiation therapy, spleen After resection, the graft-versus-host after bone marrow transplantation can be accompanied by eosinophilia, because it has a clear history and corresponding clinical manifestations, it is not difficult to identify.
