Overview: idiopathic eosinophilia syndrome (idiopathic hypereosinophilic syndrome, IHES) is a group of unknown cause, continuing high degree of eosinophil proliferation, and organ damage associated with a variety of diseases.[Cause]
caused by a common cause of this disease are:
8. others such as eosinophilic gastroenteritis and endocarditis and lymphoid granuloma. There are also unexplained eosinophils than 1500/μl and continued more than six months, accompanied by the performance of multi-organ infiltration, poor prognosis, often due to heart attack deaths. disease may be caused by this disease and see the classification of diseases (Table 1).
(B) the pathogenesis
IHES patients eosinophilic granular cytoplasm less, and normal cells with larger classes see the crystal structure, a larger proportion of fine particles cytoplasm. HES eosinophils in patients with mainly low-density cells (about 61%), compared with normal eosinophils, they increased production of leukotriene C4, antibody-dependent enhancement of parasite toxicity, and found that peripheral blood low- density the proportion of eosinophils and the degree of eosinophilia is directly related. Also found in the HES patients and normal low-density eosinophil leukotriene C4 production had increased; also proved in the blood of HES patients with increased IL-5 levels, which may be one of the reasons the incidence of HES. In addition, patients in the HES in peripheral blood mononuclear cells can be detected in IL-2R mrna expression. HES eosinophils of both normal morphology and function were also abnormal. Morphological characteristics: nuclear lobulation too. particle size than normal cells, reduce the number of specific granules, cytoplasmic vacuoles visible. HES eosinophils have cytotoxicity, antibody and complement can be combined with anti-parasite, nucleated cells. The study found that despite the HES eosinophils in ECF, and MBP levels less than normal eosinophils, but ECP release increased 83%, epo release increased 15%, which may stimulate degranulation related to the different factors. Degranulation may be due to the increase in certain cytokines, especially IL-5 induced. Also found that patients in the HES, the release of neutrophil function is not affected. eosinophilic cytoplasmic granules of toxic products cause tissue damage, such as endocardial fibrosis, nervous system disorders.[Sign]
idiopathic eosinophilia syndrome increased the early symptoms?
can be acute or chronic, can be benign or malignant. The most common clinical manifestations were fever, cough, chest pain, palpitations, shortness of breath, weakness, fatigue, weight loss, skin itching, skin rash. However, lesions involving various tissues and organs, damage to varying degrees, a variety of clinical symptoms. organ involvement is often the blood system, cardiovascular system, skin, nervous, respiratory, gastrointestinal and liver and spleen and so on. The spread of the system to the most prominent cardiovascular disease, about 80% of patients with cardiac involvement, of which half the patients had congestive heart failure, 1 / 3 of patients with organic murmur.
1975 年, Chusid and other current diagnostic criteria proposed by IHES is still commonly used in clinical:
who meet the above three conditions can be diagnosed as IHES. Diagnosed as IHES patients, the clinical manifestations of renal damage and pathological changes, side by side in addition to other autoimmune diseases and systemic diseases can be diagnosed IHES with renal damage.
reference to this disease diagnostic criteria are as follows: external blood eosinophil percentage than normal (7%) or absolute value than the normal value (0.45 × 109 / L) can be diagnosed as eosinophilic histiocytosis. according to an increase in eosinophils in the extent of clinically can be divided into light, medium and heavy three.
(2) moderate: absolute number of eosinophils (1.5 ~ 5) × 109 / L (1500 ~ 5000mm3), classified in 15% to 49 %.
idiopathic eosinophilia syndrome increased ate?[Prevent]
Once bitten by mad animals, scratched, injured as soon as possible with 20% of soapy water wash the wound thoroughly, then flush with water, cleaning and washing time not less than 20 minutes. Then with 2% -5% iodine and 75% alcohol rubbed the wound to remove or kill the local virus. Did not hurt as long as the large blood vessels, so as not to suture, do not wrap. Larger wound or facial injuries affect the face, in the debridement done after disinfection, for loose stitching and dressing, such as deep wound, the wound is large, should be placed drainage of the wound in order to facilitate the elimination of pollutants and secretions. Or the occurrence of severe trauma in the head, face, hands, neck, etc., on the basis of the above treatment, immediately after the skin test negative to do around the wound infiltration of anti-rabies immune serum injection, and must be applied before suturing the wound.
post-exposure vaccination is also important. After people have been bitten by mad animals, rabies control clinics to immediately vaccinated against rabies and to ensure full enough vaccination. Detection of antibodies after vaccination is generated, if there is no antibody or antibody titer is too low, should continue to be vaccinated. Vaccinated within one year of full and effective vaccine has been bitten by animals suspected mad, should be 0 and 3 days of the vaccinated. Full vaccination (0,3,7,14,28 days 5 pin) more than one year, was bitten by animals suspected mad, the whole vaccine should be vaccinated.
In addition, the study of rabies virus in laboratory workers, veterinarians, rabies in patients with frequent contact with medical staff, animal management staff, field staff, and no self-protection but regular contact with animals and children, can be regarded as high risk of rabies infection, pre-exposure vaccination should be carried out regularly to maintain a high level of antibodies.
present, rabies is still an inevitable fatal disease after the onset of the symptomatic treatment, including isolation of patients, to prevent saliva contamination, keep the patient quiet, reduction of light , wind, sound and other stimuli, manic when using tranquilizers.[Treat]
idiopathic eosinophilia syndrome, pre-treatment increased precautions?
1. adrenocorticotropic hormone can inhibit the production of eosinophils, the primary and secondary eosinophilia syndrome are valid, so often as the preferred treatment . prednisone 1mg / (kg · d), oral administration, the incidence may be quite acute dose of dexamethasone intravenously. Condition improved, changed to oral medication, the original amount for 2 weeks, then 2 to 3 months, reduced to half volume, then gradually reducing the maintenance of a year or so. If the reduction in the disease process over and over again, or at least should be restored to the reduction of premedication. Generally believed that these patients respond well to prednisone therapy: ① skin manifestations of angioedema; ② serum ige increased; ③ oral prednisone significantly decreased eosinophils after long duration.
2. cytotoxic significant for leukocytosis, peripheral blood cells appear in the original grains, organ infiltration, rapid progression of critically ill patients and those with poor efficacy of corticosteroid therapy may increase with hydroxyurea therapy, 1 ~ 2g / d, 7 to 14 days after treatment began to decrease white blood cells, white blood cells dropped to <10 × 109 / L, the use of low-dose maintenance (every day or every other day 0.5g). Can also be added with vincristine: 1 ~ 2mg / times a week. vincristine can be quickly reduced white blood cells, 1 to 3 days medication can decrease white blood cell count, high white blood cells is generally used for patients. vincristine treatment, but also with VP16 (VM26), alkylating agents such as chlorambucil (tumor Ning), etc.
3. immunosuppressive drugs and biological factors interferon (1.0 ~ 6.25) × 106U / d, subcutaneous injection, continuous application. cyclosporin a common dose 4mg/kg, can be combined with the use of low-dose corticosteroids.
5. splenectomy about 43% of HES patients with splenomegaly, splenomegaly and hypersplenism may occur splenic infarction, leading to anemia and thrombocytopenia. Severe hypersplenism and splenic infarction, splenectomy may be considered.
6. the rapid progression of bone marrow transplantation, conventional therapy, there are chromosomal abnormalities, younger than 50 years old, HLA-matched donor, consideration should be given allogeneic hematopoietic stem cell transplantation.
7. cardiac surgery combined heart attack, 50% of patients with mitral or tricuspid valve membrane damage, possible valve repair or valve replacement surgery. Okinawa on Lvfu viable myocardial fibrosis endocarditis endarterectomy or thrombectomy. cardiac surgery can improve the cardiac involvement of the heart function, prolong its survival.
(b) the prognosis
a variety of different causes of eosinophilic histiocytosis, significant differences between the prognosis it is necessary to identify the poor prognosis associated with eosinophilia of the disease. IHES untreated patients, 50% died within one year, the average survival of 9 months. Since the 1980s, the use of hormones, cytotoxic drugs, 5-year survival rate of 20% to 80%.
patients with early headache, anxiety, fever, wound healing abnormalities such as itching or numbness feeling. 2 to 3 days after the violent type who showed fear of water, fear of wind, fear of light. Water, wind, light reflex irritation of the throat can be seizures. Patients with nervous anxiety, fear, irritability. Condition gradually worsened, and convulsions, neck stiffness, increased salivation, fever, heart failure, respiratory paralysis. therapy can be sudden death. paralysis type mainly as limb paralysis, coma and death.[Diff]
itchy wound healing, in fact, and human skin structure and regenerative capacity of the organization. It turned out that sub-human skin epidermis and dermis layers, the dermis is the subcutaneous tissue below. In addition, the skin there is hair, sebaceous glands, sweat glands, (toe) A-level appendages. The different structure of the skin wound the feelings are not the same. Because the body's regenerative ability of various organizations, as is the ability to regenerate nerve tissue less, under normal circumstances neural fast long is good, but also a good long time the wound faster. When the nerve endings grow connective tissue, the wound can feel the itch and pain.
numbness wound healing: the wound at the nerve injury. Severe crush injury will usually damage the surrounding tissue, the skin has a certain impact of peripheral nerves, nerve injury requires a slow recovery, specifically the degree of recovery varies.
patients with early headache, anxiety, fever, wound healing abnormalities such as itching or numbness feeling. 2 to 3 days after the violent type who showed fear of water, fear of wind, fear of light. Water, wind, light reflex irritation of the throat can be seizures. Patients with nervous anxiety, fear, irritability. Condition gradually worsened, and convulsions, neck stiffness, increased salivation, fever, heart failure, respiratory paralysis. therapy can be sudden death. paralysis type mainly as limb paralysis, coma and death.[Disease]
more skin symptoms
depression depressed scars scar scar damage after contraction depressed scars itching scars old age acne scars scar formation rash maculopapular rash of the skin lesions after exposure back zhangdou nasolabial red chapped skin lichen planus calcification of epidermal keratinocytes full thickness skin necrosis and table exfoliative dermatitis pellagra-like rash
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