idiopathic eosinophilic syndrome
Introduction
Introduction to idiopathic eosinophilia syndrome Idiopathic hypereosinophilic syndrome (IHES) is a group of unexplained eosinophils that continue to hyperplasia with multiple organ damage. basic knowledge The proportion of illness: 0.0006% Susceptible people: no specific population Mode of infection: non-infectious Complications: thrombosis, congestive heart failure
Cause
Idiopathic eosinophilia syndrome
(1) Causes of the disease
Common causes of this disease are:
1. Parasitic diseases: such as aphids, hookworms and schistosomiasis infections.
2. Allergic diseases: such as bronchial asthma and urticaria.
3. Skin diseases: such as psoriasis, eczema and exfoliative dermatitis.
4. Blood diseases and tumors: such as lymphoma, eosinophilic leukemia, chronic myeloid leukemia, metastatic cancer.
5. Autoimmune diseases such as systemic lupus erythematosus.
6. Certain pulmonary-derived eosinophilia.
7. Certain drugs: such as penicillin, streptomycin, sulfonamides.
8. Others: such as eosinophilic gastroenteritis and endocarditis and lymphogranuloma, in addition to unexplained eosinophils higher than 1500 / l and lasted for more than half a year, accompanied by multiple organ infiltration The performance, poor prognosis, often due to heart disease, death, may cause the disease of the disease and see the disease classification.
(two) pathogenesis
IHES patients have less cytoplasmic granules in eosinophils and contain a larger crystal-like structure that is not seen in normal cells. The proportion of cytoplasmic fine particles is large, and eosinophils in HES patients are mainly low. Density cells (about 61%), compared with normal eosinophils, their leukotriene C4 production increased, antibody-dependent parasitic toxicity increased, and the proportion of low-density eosinophils in peripheral blood was found to be eosinophils The degree of cell proliferation is directly related, and the production of leukotriene C4 in low-density and normal-density eosinophils in HES patients is also increased; it is also shown that IL-5 levels in blood of HES patients increase, which may be the cause of HES In addition, IL-2R: mRNA expression can be detected in peripheral blood mononuclear cells of HES patients. Eosinophils of HES have both morphological and normal morphological functions, and abnormalities. Morphological characteristics: nuclear lobes Too much, the particle volume is smaller than that of normal cells, the cytoplasm is visible vacuoles, the eosinophils of HES have cytotoxic effects, the parasites that can kill antibody and complement, nucleated cells, research findings, The ECF and MBP levels in HES eosinophils were less than normal eosinophils, but the ECP release increased by 83% and the EPO release increased by 15%, which may be related to the different stimulating factors of degranulation. It may be due to the induction of certain cytokines, especially IL-5. It is also found that in HES patients, the release function of neutrophils is not affected, and the toxic products in the cytoplasmic granules of eosinophils cause tissue damage, such as heart. Intimal fibrosis, nervous system dysfunction, etc.
Prevention
Idiopathic eosinophilia syndrome prevention
The main measure is to strengthen the prevention and treatment of the induced cause to prevent the occurrence and progress of the disease.
Complication
Idiopathic eosinophilia syndrome complications Complications thrombosis congestive heart failure
1. A common complication is heart disease
Intramyocardial thrombosis and fibrosis, chordae fibrosis leads to atrioventricular valve regurgitation, eventually progressive congestive heart failure, echocardiography can be used for diagnosis and monitoring, nervous system is affected by embolism from the heart, diffuse Sexual encephalopathy and peripheral neuritis (multiple mononeuritis).
2. Combined kidney damage
In severe cases, azotemia, renal biopsy, multiple renal embolism, renal interstitial eosinophil infiltration, etc. may occur.
Symptom
Symptoms of idiopathic eosinophilia syndrome Common symptoms Kidney damage Eosinophilia Heart failure Chest pain fatigue Fatigue palpitations Shortness of breath Weight loss
May be acute or chronic, benign or malignant, the most common clinical manifestations are fever, cough, chest pain, palpitations, shortness of breath, fatigue, fatigue, weight loss, itchy skin, rash, etc., but due to the different tissues and organs involved in the lesion, Different degrees of damage, clinical symptoms are also diverse, the affected organs often have blood system, cardiovascular system, skin, nerves, respiratory system, gastrointestinal tract and liver spleen, etc., the cardiovascular system The most prominent lesions, about 80% of patients with heart involvement, of which half of the cases have congestive heart failure, and 1/3 of patients have organic murmur.
Examine
Examination of idiopathic eosinophilia syndrome
1. Blood: elevated eosinophils in peripheral blood, abnormal morphology of eosinophils, eosinophils >8% in white blood cell classification, absolute value of eosinophils >0.4×10 9 /L, peripheral blood smear Immature myeloid cells can be seen, with mild anemia and abnormal platelet count.
2. Bone marrow: active bone marrow hyperplasia, active granulocyte proliferation, granulocyte promyel, middle granule, late granule, rod-shaped nucleus, eosinophils can be found in the nucleus stage.
3. Parasitic diseases: fecal egg test and parasite skin allergy test positive.
4. Sputum examination: pulmonary performance is the main: can be used for eosinophilic examination.
5. Urine test: Renal damage syndrome can occur in patients with kidney damage, urine urine and hematuria can be seen in urine tests.
6. Electrocardiogram: There may be ST-T changes, conduction block, etc., echocardiography shows left ventricular free wall and interventricular septum thickening.
7X line examination: there may be lung infiltration shadow, mainly pulmonary interstitial infiltration, a small number of patients may have pulmonary infarction.
8. EEG: There may be abnormalities.
9. Tissue biopsy: multiple renal embolism, renal interstitial eosinophils and lymphocytic infiltration, mesangial mesangial hyperplasia, basement membrane thickening, liver biopsy showing eosinophil infiltration, small intestinal biopsy can be found The lamina propria eosinophil infiltration, skin and muscle biopsy showed perivascular and interstitial eosinophil infiltration.
Diagnosis
Diagnosis and differentiation of idiopathic eosinophilia syndrome
diagnosis
In 1975, the diagnostic criteria for IHES proposed by Chusid et al. are still commonly used in clinical practice:
1. The peripheral blood eosinophils increased significantly (>1.5×109/L) and lasted for more than 6 months.
2. Multiple system organ damage occurs, no other reason can be explained.
3. Can not find a common cause of eosinophilia.
Those who meet the above three conditions can be diagnosed as IHES. Patients diagnosed with IHES have clinical manifestations and pathological changes of renal damage, and exclude other immune diseases and systemic diseases, and can diagnose IHES with renal damage.
The reference diagnostic criteria for this disease are as follows: the percentage of eosinophils in the external blood exceeds the normal value (7%) or the absolute value exceeds the normal value (0.45×109/L) to diagnose eosinophilia. The degree of acid granulocyte increase can be divided into three levels: light, medium and heavy.
(1) Mild: The absolute number of eosinophils is less than 1.5 × 109 / L (1500 / mm3), accounting for less than 15% in the classification of white blood cells.
(2) Moderate: The absolute number of eosinophils is (1.5 to 5) × 109 / L (1500 ~ 5000 mm3), accounting for 15% to 49%.
(3) Severe: The absolute number of eosinophils is greater than 5×109/L (5000 mm3), accounting for 50% to 90% of the classification.
Differential diagnosis
The disease should be differentiated from various reactive eosinophilia and eosinophilic leukemia.
Chronic myeloid leukemia
The disease is insidious onset, with a long course of disease, characterized by splenomegaly. Except for eosinophilia in peripheral blood, there are increased basophils, and neutral and young granules in the bone marrow. Cell growth is predominant.
2. Eosinophilic leukemia
It can be seen that the peripheral blood leukocytes are significantly increased, and there are naive eosinophils, and the bone marrow hyperplasia is extremely active. The classification is dominated by immature eosinophils, and the clinical manifestations are similar to acute leukemia.
