Clinical and Pathological Features of Pulmonary Intravascular Lymphoma

  Objective  To investigate the clinical and pathological features of pulmonary intravascular lymphoma (IVL).

  Methods  The clinical, radiographic, and pathological features of five cases of pulmonary IVL diagnosed by lung biopsy from March 2008 to June 2011 were retrospectively analyzed, and similar cases reported in English literatures were reviewed.

  Results  These 5 patients (3 males and 2 females) aged 36 to 59 years, with a median age of 45 years. The chief complaints included fever (5/5), weight loss (5/5), cough (4/5), fatigue (3/5), and dyspnea (2/5). Pulmonary function tests mainly showed some degree of decreased diffusing capacity. All the five cases had high serum lactate dehydrogenase (range:316-1025 U/L). Chest CT showed diffuse ground-glass opacity (n=3), bilateral multiple nodular density with subpleural wedgeshaped density and thickening of bronchial wall (n=1), and consolidations in the right lower lobe (n=1). On histopathology, single or clustered lymphoma cells were seen within the lumens of capillaries of the alveolar septum, with the alveolar structure remained; the lesion was small and could not be easily identified. Pulmonary infarction caused by tumor thrombus was identified in one case. Immunophenotyping showed B cell lineage in 3 cases and T cell lineage in 2 cases. During the follow-up period (range:0.67-6 months; mean:3.17 months), 3 patients with B cell pulmonary IVL received R-CHOP chemotherapy and their diseases were improved and remained stable. One patient with T cell pulmonary IVL received CHOP chemotherapy but died due to gastrointestinal bleeding and respiratory failure 20 days after diagnosis. One patient with T cell pulmonary IVL was lost to follow-up.

  Conclusions  Pulmonary IVL is a rare malignant disease that mainly seen in the elderly. Its clinical symptoms are not specific. Chest CT usually reveals diffuse interstitial changes, which should be carefully differentiated from interstitial pneumonia, pulmonary infection, and lung metastatic cancer. It can be pathologically diagnosed through transbronchial lung biopsy or surgical lung biopsy.