Clinicalanalysis Of Nine Cases Of Adult Pulmonary Langerhans Cell Histiocytosis

Objective:To analysis the clinical materials of 9 cases of adult pulmonary langerhans cell histiocytosis in order to arouse the awareness of the disease and reduce the cases of missed diagnosis and misdiagnosis.Method:A retrospective analysis of 9 cases of adult pulmonary langerhans cell histiocytosis which proven in the first affiliated hospital of Guangxi Medical University during the February,2004 to November,2014.Then the clinic,pulmonary function, iconography, pathology, treatment will be analyzed combining with the discussion of domestic and international literatures.Results:①9 patients were adult pulmonary langerhans cell histiocytosis.Among them, 3 cases were pulmonary langerhans cell histiocytosis(which referred to as PLCH),age 18-27,3 cases were all male;6 cases were multisystem of langerhans cell histiocytosis(MS-LCH)),age 17-71,average age was(29.67±20.65).4 cases(4/9)had a history of smoking. Most of the cases had none of chronic diseases. ②Clinical characteristics : The most common respiratory symptoms were cough(8/9) 、 expectoration(8/9) 、 dyspnea(6/9);spontaneous pneumothorax(5/9). The nonspecific systemic symptom was theloss of weight(4/9),and none had the symptoms such as fever,night sweat and so on. In the chest signs,4 cases with pneumothorax,2 cases with crackles, 2cases with P2 hyperthyroidism and so on. None got Velcro sound. None of the 3cases of PLCH had extrapulmonary symptoms and signs. Among the 6 cases of MS-LCH,there were diabetes insipidus(2/6),abdominal pain(1/6), ostealgia(1/6), hearing loss(1/6)and so on that found outside the chest. Besides,extrapulmonary physical signs such as abdominal tenderness(1/6),skin rash(1/6),derma and sclera stained yellow(1/6)and so on could be seen. None got clubbing. ③Imaging characteristics:The computed tomography(CT) scan of the chest provided radiographic findings,such as pulmonary cysts(8/9),grid shadow(6/9),increased transparency of the lung field(5/9),pneumothorax(4/9),nodules(3/9),distributed predominantly in upper and mid-lung zones.④Pulmonary function:pulmonary function test findings were variable,obstructive ventilation disorder 2 cases(2/9),mixed ventilation disorder 6 cases(6/9), diffusion(6/9)abnormality could be seen.⑤Laboratory and equipment examination : 3 cases of PLCH got no abnormity of the hepatic and renal function,blood glucose,coagulation function,myocardial enzyme and so on;2 cases( 2/3) of the total number of white blood cells and the acidophilic granulocyte percentage in blood routine examination increased. The 6 cases of MS-LCH got abnormity of hepatic function(4/6),blood lipid(1/6),thyroid function(1/6)and so on. What’s more,there were abnormities of liver ultrasound(3/6),thyroid ultrasound(1/6),skeleton ECT(1/6),pituitary gland MRI and so on. None got abnormity of autoantibodies. ⑥Pathology:The pathological langerhans cells could be seen from the lung tissue biopsy specimens with HE staining,which were positive for CD1 a,CD68,S-100 histochemical staining.⑦Treatment and prognosis:3 cases of PLCH received glucocorticoid treatment,3 cases improved. Among the 6 cases of MS-LCH,5 cases(5/6)received glucocorticoid treatment,2 cases 2/6)received immunosuppressor treatment,1 case(1/6)rejected treatment;After glucocorticoid treatment for pulmonary lesions,2 cases(2/6)improved,2 cases(2/6)exacerbated,1 case(1/6)stabilized,for extrapulmonary lesions,2 cases(2/6)exacerbated,2 cases(2/6)improved;2 cases(2/6)died.