Het intravasculair lymfoom; een zeldzame, maar curabele oorzaak van interstitiële longafwijkingen

- A 69-year-old man presented with dyspnoea, cough and diffuse interstitial changes and ground-glass effect on a CT-scan. After 5 months the patient was diagnosed with pulmonary intravascular lymphomatosis. A histologic diagnosis was made by a video-assisted thoracoscopic surgical biopsy. Treatment with combination chemotherapy, i.e. 8 cycles of cyclophosphamide-doxorubicin-vincristine-prednisone (CHOP), resulted in a complete remission and a disease-free survival of 5 years at the last follow-up. Intravascular lymphomatosis is a rare but curable cause of interstitial lung disease. Intravascular lymphomatosis is an uncommon type of non-Hodgkin’s lymphoma, characterized by proliferation of neoplastic lymphoid cells within the lumen of small blood vessels, resulting in thrombotic and ischaemic complications in multiple organ systems. Primary pulmonary presentation is even more uncommon. It is important to consider intravascular lymphomatosis in the differential diagnosis of unexplained interstitial lung disease, since early diagnosis and treatment may lead to complete remission and long-term survival.

Ned Tijdschr Geneeskd 2005;149:1118-22