| Purpose : To explore the clinical characteristics of diffuse pulmonary lymphangiomatosis(DPL).Methods : The clinical manifestations,image characteristic,pathological features and treatment of 1 case of diffuse pulmonary lymphomatosis admitted by our hospital were collected,and combined with the literature review to discuss.Results: The patient was a young male,the onset age was when he was 18,and the main clinical manifestations were cough,sputum cough and dyspnea.Chest CT revealed diffuse patchy shadows of the two lungs with pleural and pericardial effusion and mediastinal mass.Pleural effusion is chylous.The histopathology of the pericardium is mainly in the form of cavernous lymphangioma,and the immunohistochemical results are part D2-40(+),part CD31(+),part CD34(+),part CD20(+),and a few parts of CD3(+).In April 2012,her condition was improved after subtotal pericardial resection,pleural puncture,and anti-infective treatment.However,it still has recurring attacks,and it has improved after infection and symptomatic treatment.The oral prednisone treatment was given in November 2017.After 2 months of treatment,the patient discontinued prednisone on his own.After 4 months of follow-up,no recurrence occurred.Literature review result: "lung+ lymphangioma disease" as keywords through China knowledge Network and Wanfang Database Retrieval of documents from 1990 to 2018,received the relevant cases of 7 reports,10 cases were reported with clear pathological diagnosis.Using "diffuse pulmonary lymphangiomatosis" as the key word,32 cases were reported by Pup Med,39 cases(49 cases)were reported with definite pathological diagnosis.A total of 49 cases of.Among the 49 patients,male: female was 1.5: 1,the onset age was 8 months to 55 years old,the average age was 17.7 years old,the median age was 13 years old.Clinical manifestations without symptoms 6%(3/49),breathing difficulties 69 %(34/49),cough 49 %(24/49),expectoration 24 %(12/49),hemoptysis 20%(10/49),pyrexia 16%(8/49),dyskinesia 12%(6/49),chest distress 10%(5/49),chest pain 8%(4/49),other cases of abdominal pain,low back pain,palpitations of the heart and swelling of the lower extremities are individual cases.Chest imaging always showed the thickening of interlobular septum between the two lungs.And it is mostly scattered in distribution Strip shadow,Grid shadow and Patch shadow.Most patients have pleural effusion and mediastinal low density.Patients will have pericardial effusion when attacking pericardium.The diagnostic mode were surgical biopsy 55%(27/49),thoracoscopic biopsy23%(11/49),transbronchial lung biopsy(TBLB)16%(8/49),mediastinal biopsy 4%(2/49),and autopsy 2%(1/49).The pathological findings showed that the lesions were polygenic,with diffuse proliferation and dilated lymphatic vessels,proliferating lymphatic vessels,no abnormal endothelial cells,and various degrees of fibrosis.Some of the hemoglobin macrophages were found.The immunohistochemical staining was D2-40 positive.Treatments include surgery,radiotherapy,diet,medication,and so on.Of the 49 patients,11 did not mention the prognosis.In the remaining 38 cases,a total of 16 patients died,accounting for 42 percent,from January to December,with an average duration of 35.8 months.Conclusion: 1.Diffuse pulmonary lymphangiomatosis is rare in clinical practice and is more common in children than in women.Its clinical manifestation lacks specificity.The lesions are invasive,often involving the lungs,mediastinum,pleura,pericardium,etc.,causing dyspnea,respiratory failure and death.2.The main chest CT(computed tomography)findings of diffuse pulmonary lymphangiomatosis were thickening the interlobular septum of two lungs and appearing diffuse funicular shadows,reticular opacity and pathcy shadows.Most patients had pleural effusion and mediastinum space occupying in low density.No obvious enhancement after enhancing chest CT except for hemangioma.Bronchoscopy and pulmonary function test have no specificity.3.Diagnosis depends on pathology.Pathologically manifested as lymphangioma,diffuse proliferation and expansion of lymphatic vessels,lymphatic endothelial cells proliferation and differentiation,no abnormality,immunohistochemical staining D2-40 positive.4.Pathology is mostly obtained through surgery,and TBLB,thoracoscopy,etc.are also commonly used methods.5.It was found that DPL was not specifically treat,that prognostic difference of DPL was high,the mortality was high,and the earlier the age of pathogenesis,the worse the prognostic. |
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