INTRAVASCULAR LYMPHOMATOSIS - A CLINICOPATHOLOGICAL STUDY OF 2 CASES PRESENTING AS AN INTERSTITIAL LUNG-DISEASE

Aims: Intravascular lymphomatosis is an uncommon type of non-Hodgkin's lymphoma characterized by intravascular proliferation of neoplastic l ymphoid cells. Although the tumour is basically a systemic disease, ev entually involving multiple organs, primary presentation in the lung i s rare. Methods and results: We describe the clinicopathological featu res of two patients with intravascular lymphomatosis presenting in the lung. One patient complained of fever, headache and chest pain; the o ther, of dyspnoea on exertion and headache. Both patients showed retic ulonodular density on chest radiography and decreased diffusion capaci ty. Lung biopsy showed features characteristic of intravascular lympho matosis. Malignant lymphoid cells were CD30 positive T-cells of anapla stic large cell type in one patient and B-cells of large cell type in the other. There was a poor response to chemotherapy and both patients died of the disease within 3 months of diagnosis. Conclusions. These cases and 10 previous reports illustrate the need to include intravasc ular lymphomatosis in the differential diagnosis of interstitial lung disease.