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Objective: A retrospective analysis of Langerhans cell histiocytosis (LCH) clinical features, diagnosis and treatment process, to increase awareness of the disease, accurate diagnosis and treatment, in order to improve the cure rate, thereby improving the quality of life of children. Methods: A retrospective analysis from January 2002 to December 2010 Children's Hospital of Chongqing Medical University hospital 62 cases of LCH income children with clinical data. Summarize their age, gender differences, regional differences, seasonal changes, clinical manifestations, laboratory tests, imaging, pathology, diagnosis and treatment and prognosis. Result: 1.62 cases are in accordance with Zhang Nan \Initial diagnosis in 40 cases, diagnosed three cases, diagnosed 19 cases. Le - Gaucher disease 31 cases, South Korea - XUE - Ke disease in 10 cases, 21 cases of eosinophilic granuloma. 2.62 cases in the youngest patients a month, the oldest patients 15 years old in March, with a median age of 14 months. lt; 1-year-old 25/62 cases (40.3%), 1-2 years in 16/62 cases (34.1%), 2-3 years old 5/62 cases (8.1%). Less than 3 years old 46/62 cases (74.2%), more common in infants and young children. 62 cases of 34 cases of children with male children, female patients 28 cases, male to female ratio 1.21:1,1 years of age and 4 years of age of onset no gender differences. 3.62 cases of patients with onset of spring in 15 cases, the onset of summer in 23 cases, 14 cases with onset in autumn and winter onset in 10 cases, the onset of summer slightly higher than other seasons 4.62 cases of patients, 41 cases from Chongqing, 16 cases from Sichuan, two cases from Guizhou, one case from Henan, one case from Hunan, one case from Fujian. Rural children with 40 cases, 22 cases of urban children, rural and urban children incidence ratio of 1.86:1. 5.62 cases in six cases with thalassemia, autoimmune hemolytic anemia, one case, Rathk's cyst two cases. 6.62 cases, 23 cases have misdiagnosed history, misdiagnosis rate of 37.1%. The most common misdiagnosis is skin disease, otitis media, lymphadenitis and other blood diseases. 7.62 cases in 32 cases of fever, rash 34 cases (with hemorrhagic, seborrheic eczema type rash mainly bleeding rash five cases, Confucius nettle rash one case, one case of scabies rash, heat rash-like rash one case, repeated batches, torso hairline more common), liver and spleen and / or lymph nodes in 31 cases, 43 cases of anemia, respiratory symptoms in 13 cases, 17 cases of ear overflow, proptosis three cases, 8 cases of diabetes insipidus , skeletal manifestations 34 cases (mainly in head mass, six cases have limited mobility), 3 cases of swollen gums, tooth loss in 2 cases, 9 cases of diarrhea (bloody three cases), edema 6 cases, one case of jaundice. 8 blood analysis 62 cases, anemia 43 cases (29 cases of severe anemia), platelets lt; 100 * 10 ~ 9 19 cases, WBC lt; 4 * 10 ~ 9 2 cases 9 58 cases of liver function tests, including protein decreased in 27 cases, 11 cases of elevated bilirubin (direct bilirubin), elevated aminotransferases seven cases. 58 cases of renal function tests were normal. Urine four protein examination three cases, one case of elevated albumin. 24-hour urinary protein excretion check one case, one case of elevated protein. Addis count check one case, the red blood cells increased. 10 immunoglobulin examination in 13 cases, 12 cases of elevated immunoglobulin (IgA increased 10 cases, IgG increased six cases, IgM increased six cases, IgE increased in 2 cases), one case of IgG, IgA, IgM were reduced. Lymphocyte classification check six cases, CD19 ~ cells increased six cases, CD4 ~ cells decreased three cases, CD8 ~ cells increased and decreased in 1 case, CD4/CD8 positive cells decreased in 2 cases. Autoantibodies checking two cases, one case of anti-nuclear antibody and anti-ss-DNA antibodies. Anti-human globulin examination in 8 cases, 1 case of direct and indirect anti-human globulin were positive. 11. CMV, EBV virus antibody test in 26 cases, 23 cases of the virus antibody positive, CMV antibody positive 17 cases, EBV antibody positive nine cases (CMV and EBV antibodies were positive in 3 cases). 12 bone marrow aspiration cytology in 61 cases, one case found a small amount of tissue cells, I showed irritation or bone marrow hyperplasia. Bone marrow chromosome 33 cases, ectopic chromosome, deletion of one case. 13 chest radiograph in 57 cases, 24 cases of abnormalities, mainly interstitial changes, one case was patchy and small nodules. 14 bone X-ray examination of 59 cases (52 cases of skull, torso bone in 51 cases, 52 cases of limb bones, pelvic bone 49 cases), 40 patients with bone destruction (skull 29 cases, 16 cases of bone torso, limbs 16 cases, 11 cases of pelvic bone), multiple parts of the bone damage bone lesions more common than individual parts. Common sites of bone destruction were the skull, femur, vertebrae, ribs, ilium, humerus. Sella destroy four cases (2 cases with diabetes insipidus, 2 patients without diabetes insipidus). 15 CT or MRI examination in 26 cases, intracranial lesions in 2 cases, 1 case of triangular area located in the left ventricle, one case in occipital gray matter junction area. Left lateral triangle one case was intracranial surgical biopsy confirmed LCH infiltration, and the other one case outstanding biopsy. Pituitary imaging abnormalities nine cases (four cases have diabetes insipidus, five cases without diabetes insipidus). 16 Pathological examination 62 cases (28 cases imprints rash, skin biopsy in 5 cases, 26 cases of bone biopsy, lymph node biopsy in five cases, three cases of mass biopsy, biopsy material or ear overflow smears 5 cases), 62 cases were find a large tissue cell infiltration (partly through two more times to find pathological tissue infiltration basis). Immunohistochemistry in 22 cases, of which 22 cases positive S100, S100 positive in 22 cases while CD1α positive 19 cases, CD1α positive 19 cases in one case did find Birbeck granules electron microscopic examination. 17. Treatment: After surgery and chemotherapy, 35 patients (LS 9 例, HSC 7 例, EGB 19 cases), of which 6 cases of single parts of the bone lesion surgery alone, 29 cases taking chemotherapy (six cases of eosinophilic granuloma also surgical treatment). Chemotherapy regimen for taking LCH-Ⅲ programs, improved DAL-HX83/90 programs, improved LCH-Ⅰ program. Were combined with anti-diabetes insipidus diabetes insipidus treatment. Followed up 27 cases (follow-up of 6-46 months, with an average follow-up of 18.1 months), 6 cases were cured (LS 1 例, EGB 5 patients), improved in 19 cases (LS 5 例, HSC 3 例, EGB 11 cases), deterioration of 2 Example (HSC 1 例, EGB 1 patients). Cure rate of 22.2%, 70.4% improved, deteriorated 7.4%. Patients were followed three cases of diabetes insipidus (follow-up period 6-40 months) still diabetes insipidus. Conclusions: 1. LCH seen in all age groups of children with different types of diseases have different good age. Our data show Le - Gaucher disease occurred in the two years of age; Korea - XUE - Ke disease occurred in the 1-4 years; eosinophilic granuloma more common in older children, but can occur in early May. (2) The data show that male and female incidence ratio of 1.21:1 LCH, male incidence slightly more than women, but a ratio of 0.92:1 age of onset was no difference between men and women, consistent with the literature; 4 years old male and female incidence ratio of 0.8:1 was no difference with reported in the literature are inconsistent, may differ from the data in children older than 4 years on the small sample size. 3. LCH geographical spread of rural and urban children incidence ratio of 1.86:1, the incidence of children in rural areas than urban children is high. (4) The disease most commonly involved system is the skeleton, skin, liver, spleen and lymph nodes, blood, lungs, skin rash, bone damage, lung disease and liver and spleen, lymph nodes, anemia is the clinical features of this disease. 5 The typical rash have diagnostic significance, especially Le - Gaucher disease; atypical rash should be OK looking skin pathology diagnosis. 6 64.5% of patients with bone destruction, including Le - Gaucher disease may also be associated with bone destruction, and many parts of the bone damage bone damage more than a single site. Therefore, the line should be suspected in patients with systemic LCH bone X-ray examination. 7 Nearly 40% of patients with pulmonary radiographic abnormalities (mainly interstitial changes), it should be suspected in patients with LCH routine chest X-ray examination. 8 pituitary imaging abnormalities without diabetes insipidus should pay close attention to the occurrence of diabetes insipidus, intracranial space-occupying lesions of LCH patients should be alert to the disease involving the central nervous system may be. 9 through multi-site rash imprints and (or) lymph node biopsy, bone biopsy histopathology can find a large number of tissue cells and other pathological diagnosis, conditions should line S-100, CD1α such as immunohistochemistry and electron microscopy to find Birbeck granules do diagnosis and diagnosis. 10 Our data show that the disease has a cell-mediated immunity, humoral immune abnormalities. Moreover, CMV, EBV infection rates as high as 88.5%. Therefore, attention should be OK immune function tests and viral antibody testing to help reveal the cause. 11 types of chemotherapy can improve the patient's condition, if early diagnosis and timely treatment also cure typing possible. Eosinophilic granuloma in addition to surgery, especially in patients with impaired bone parts should be supplemented in order to improve the efficacy of chemotherapy. After difficult to cure diabetes insipidus.
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