Pohl W
II. Medizinische Abteilung des Pulmologischen Zentrums der Stadt Wien.
Wien Klin Wochenschr. 1996;108(15):473-7.
Idiopathic bronchiolitis obliterans organizing pneumonia (idiopathic BOOP) and idiopathic CD4+ T lymphocytopenia are syndromes of unknown origins which have been characterized in recent years. An important feature of both syndromes is the good prognosis. The clinical and histological features of idiopathic BOOP are unspecific but characteristic, whereas the clinical features of idiopathic CD4+ T lymphocytopenia are not homogeneous. We present a report on the first patient in whom both syndromes were found simultaneously. Initially the chest x-ray showed bilateral patchy opacities and the peripheral blood showed a severe CD4+ T cell depletion. The diagnosis of BOOP was verified by open lung biopsy, which demonstrated the histological features of BOOP. No evidence was found of congenital or acquired reasons associated with CD4+ T cell depletion or with the histological features of BOOP, which implies the diagnosis of idiopathic CD4+ T lymphocytopenia and idiopathic BOOP. Within a follow-up period of 14 months the pulmonary opacities disappeared completely and the CD4+ T cells increased, but did not reach normal values.
特发性闭塞性细支气管炎伴机化性肺炎（特发性BOOP）和特发性CD4 + T淋巴细胞减少症是近年来已被明确的不明原因综合征。这两种综合征的一个重要特征是预后良好。特发性BOOP的临床和组织学特征不具有特异性但具有特征性，而特发性CD4 + T淋巴细胞减少症的临床特征并不一致。我们报告了首例同时发现这两种综合征的患者。最初胸部X线显示双侧斑片状阴影，外周血显示严重的CD4 + T细胞减少。经开胸肺活检证实为BOOP，显示出BOOP的组织学特征。未发现与CD4 + T细胞减少或BOOP组织学特征相关的先天性或后天性原因，这意味着诊断为特发性CD4 + T淋巴细胞减少症和特发性BOOP。在14个月的随访期内，肺部阴影完全消失，CD4 + T细胞增加，但未达到正常值。
