[Fever and nonspecific pulmonary lesions--a rare differential diagnosis]

A 70-year-old woman presented with rapidly increasing weakness, night sweats, progressive dyspnea and daily fever up to 39 C. 30 years before early stage breast cancer had successfully been treated by resection.

Blood tests revealed moderate normocytic anemia and a considerable elevation of the serum level of lactate dehydrogenase (LDH). The differential blood count was normal. Chest radiography showed interstitial infiltrates. Computed tomography revealed reticular shadows and ground-glass opacities which on histological examination were attributed to intravascular lymphoma (IVL) of B cell lineage.

Treatment with eight cycles of the R-CHOP 14 regimen (rituximab, cyclophophamide, doxorubicine, vincristine, prednisone; interval: 14 days) resulted in complete remission with rapid resolution of constitutional symptoms. 7 months later the IVL relapsed, again with fever and an elevation of the LDH level. Bone marrow biopsy revealed intravascular infiltration by lymphoma cells. Salvage treatment with six courses of a methotrexate-containing protocol led to a second complete remission.

Nonspecific constitutional symptoms, a large variety of potential clinical presentations and the infrequency of the disease render the ante mortem diagnosis of an intravascular lymphoma difficult. Treatment follows the same principles as in nodal aggressive non-Hodgkin's lymphomas.