| Objective:Analyze the clinical features, diagnosis and therapy of a case of pulmonary alveolar proteinosis(PAP) secondary to idiopathic thrombocytopenic purpura(ITP) and summarize the experience of diagnosis and therapy, to improve the strategy of the diagnosis and therapy of PAP secondary to ITP, cause the attention of clinician.Method:Retrospectively review of a case of PAP secondary to ITP administrated in the first affiliated hospital of Guangxi Medical University in2011, analyze its clinical manifestation, pulmonary function, radiological features diagnosis and therapy.Result:(1)General information:the Patient was male, aged23, farmer;(2)Clinical manifestations:Repeatedly gingival bleeding, coughing and dyspnea, mild cyanosis, moist crackles in down lung field;(3)Laboratory examination:there were severe hypoxemia, white blood cell count and neutrophil percentage rate elevating, platelet decreasing and abnormal immunity index;(4)The radiological features of the lung:two lungs have a diffuse pattern of ground-glass opacities and septal reticulations with indistinct borders, which shows a lower zone predominance, air bronchogram could be seen in some ground-grass opacities. Opacities have a typically geographic distribution, with juxtaposition of health and sick zones. Two sides of pleura have a local thickening;(5) Pulmonary function:mild restrictive ventilatory dysfunction, moderate diffuse dysfunction;(6) Bone marrow cytology:There is a mild and abnormal hematopoiesis in megakaryocytic, with a decreased production, ITP could not be excepted;(7)Bronchoalveolar lavage fluid:periodic acid-schiff (PAS) stain positive;(8) Transbronchial lung biopsy:Alveolar accumulation of light red fibrinoid agglutination, less inflammatory cells, thickened alveoli septum, PAS stain positive.Conclusion:(1)PAP secondary to ITP is rare, when an ITP patient has a cough, fever or dyspnea, especially anti-infective therapy is poor, we should think of the possibility which PAP secondary to ITP.(2)Secondary PAP is associated with the underlying diseases or toxic exposures.(3)The chest high resolution computerized tomography(HRCT) of PAP shows characteristic presentations, such as ground-glass opacity, septal reticulation and geographic distribution, but these also could be seen in other diseases, we should make a differential diagnosis combine with clinical manifestation.(4)It is easy to get infection, once appear severe dyspnea or fever, we should complete related examinations to confirm pathogens. Early and fully therapy treatment can improve prognosis.(5)Compare with idiopathic PAP, secondary PAP’s curative effect of whole-lung lavage is worsen, which reminds us that emphasizing the therapy of underlying diseases is more important. |
