Intravascular lymphoma: two case reports demonstrating the heterogeneity of the disease

摘要

Intravascular lymphoma (IVL) is a rare subtype of extranodal large-B-cell lymphoma, histologically characterized by accumulation of clonal lymphocytes in small vessels of different organs. Overall survival is usually poor. The clinical manifestations are highly variable, depending on the involved organs. Cutaneous and neurological involvement is frequent; the cutaneous symptoms are heterogeneous with erythema, erythematous papules and plaques, generalized telangiectases and lesions resembling panniculitis or Kaposi sarcoma. IVL may also be limited to the skin; this entity is included the current classifications. We present here two patients with two different variants of IVL. One showed only skin involvement with panniculitis-like induration and responded well to therapy with Rituximab-CHOP (Rituximab plus Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone). The other patient had distinctive cutaneous and neurological symptoms which did not respond to therapy. By means of these two patients, we present the heterogeneity of IVL and discuss current aspects of diagnosis and treatment.