ObjectiveTo highlight the characteristics of secondary pulmonary alveolar proteinosis (PAP) associated with malignant hematological diseases. MethodsThe clinical data of three patients with secondary PAP were analyzed and the related literature was reviewed. ResultsThree patients were diagnosed with secondary PAP by exclusion of primary or autoimmune PAP and denied the history of inhalation of occupational dusts. Two patients with secondary PAP were associated with chronic myelocytic leukemia, and the third one was associated with myelodysplastic syndrome. The performance on HRCT of the PAP associated with hematological malignancy was different from the primary PAP. Three patients were pathologically diagonised by brochoalveolar lavage fluid. One patient was successfully treated with inhalation of granulocyte-macrophage colony-stimulating factor (GM-CSF). ConclusionsSecondary PAP associated with hematological malignancy is very rare. The untypical HRCT is the main cause of misdiagnosis. Some patients may benefit from GM-CSF theatment.
ObjectiveTo observe the diagnostic sensitivity and specificity of color Doppler flow imaging (CDFI) for persistent hyperplastic primary vitreous (PHPV). MethodsThe clinical data of 71 children (93 eyes) with congenital cataract which suspected of concurrent PHPV were retrospectively analyzed. The children included 45 males (54 eyes) and 26 females (39 eyes), aged from 1 to 24 months, with an average age of (7.6±4.3) months. All eyes were examined by CDFI, and observe whether there was a pathological strip in the vitreous, the site of the connection between the strip echo and the wall of the eye and the signal of blood flow on the strip echo. Within 1 week after a CDFI examination under general anesthesia, 64 children (79 eyes) underwent lens excision combined with vitrectomy and the other 14 eyes of 7 children underwent mydriatic fundus examination by two experienced eye specialists. Combined with clinical features of PHPV, the diagnosis and differential diagnosis were made according to the clouding of the lens, posterior capsule proliferation, vitreous proliferation and retinal detachment position which were found during operation and fundus examination. Compare and analyze the CDFI examination results and the above diagnosis results, calculate the sensitivity and specificity of CDFI for PHPV. ResultsIn surgery and mydriatic fundus examination results of 93 eyes, vitreous abnormal in 85 eyes, no significant changes were found in vitreous of the other 8 eyes. In 85 eyes of abnormal vitreous, 68 eyes were diagnosed as PHPV, 16 eyes were diagnosed as familial exudative vitreoretinopathy (FEVR), and 1 eye was diagnosed as treactional retinal detachment. In 85 eyes of abnormal vitreous which were found by surgery and fundus examination, CDFI confirmed 81 eyes and its diagnostic sensitivity was 95.3%; the other 4 eyes were not found vitreous abnormality, and the missed diagnosis rate was 4.7%. Surgery and mydriatic fundus examination found no vitreous abnormal in 8 eyes, but CDFI explored strip low echo connected with the optic disc or posterior lens capsule in vitreous. In the 68 eyes of PHPV which were diagnosed by surgery or fundus examination, 59 eyes had the same diagnosis of CDFI, the sensitivity of CDFI was 86.8%; PHPV was not diagnosed in 25 eyes by surgery or fundus examination, but only 8 eyes were also not diagnosed by CDFI, and the specificity of CDFI was 32.0%. The remaining 17 eyes were diagnosed as FEVR in 16 eyes and traction retinal detachment in 1 eye after surgery or mydriatic fundus examination, but they were all diagnosed as PHPV in CDFI. The misdiagnosis rate of CDFI was 68.0%. ConclusionFor PHPV, the diagnostic sensitivity and specificity of CDFI are 86.8% and 32.0%, respectively.
ObjectiveTo investigate the clinical, radiographic characteristics and differential diagnosis of pulmonary Langerhans cell histiocytosis (PLCH) mimicking metastasis of cancer in radiography. MethodsClinical data of 2 patients with PLCH manifesting as metastatic cancer on HRCT and PET/CT were retrospectively analyzed. Patients reported as PLCH on WanFang Database, China Knowledge Resource Integrated Database and Pubmed were reviewed to screen misdiagnosis literature and further analyzed the clinical and radiographic characteristics. ResultsTwo cases both presented with cough and sputum. 18F-FDG PET/CT showed increased 18F-FDG up-take in both nodules in the lungs. One patient presented with multiple nodules, diffuse multiple cystic changes in lungs and osteoclasia in the right 4th rib on HRCT who was diagnosed by a video-assisted thoracoscopic biopsy of rib biopsy. The other patient presented with diffuse multiple nodules on HRCT who was diagnosed by a video-assisted thoracoscopic biopsy of lung biopsy. The pathological characteristics of both biopsy specimen demonstrated infiltration by Langerhans cells (LC) and eosinophils. The LC were positive for CD1a. Literature review found seven PLCH cases who were misdignosed as depression, eosinophilic pneumonia, interstitial lung disease involvement of autoimmune disorders and malignant tumor. ConclusionWhen clinician faced with a patient suspected as metastatic cancer by HRCT and PET/CT, it is reasonable to consider PLCH as a differential diagnosis and obtain the pathological information as soon as possible so that better prognosis can be achieved through early intervention.