Disorders of the Lymph System

B-cell lymphoma with febrile inflammatory lymphoedema of the lower limbs and lower back

Ann Dermatol Venereol. 2008 Apr

Service de dermatologie, groupe hospitalo-universitaire Carémeau, rue du Professeur-Robert-Debré, 30900 Nîmes, France.

BACKGROUND: Intravascular lymphomas are diffuse large-cell lymphomas belonging to a group of high-grade non-Hodgkin's lymphomas and are generally of phenotype B. They are rare and carry a severe prognosis. Clinical polymorphism is dominated by neurological and cutaneous involvement.

PATIENTS AND METHODS: We report the case of an 80-year-old woman with cutaneous intravascular B-cell lymphoma as revealed by an isolated episode of febrile bilateral inflammatory lymphoedema. Following combined chemotherapy with rituximab and mini-CHOP (cyclophosphamide, adriamycin, oncovin and prednisone), complete remission was obtained rapidly, with no relapse at two years.

DISCUSSION: Diagnosis of these tumors is rendered difficult by the clinical polymorphism and multifocal nature of lymphocytic proliferations. In the present case, diagnosis was based on histology results since presentation of the disease in the form of bilateral inflammatory oedema of the lower limbs is not sufficient to establish lymphoma. Combined rituximab and polychemotherapy comprising a CHOP regimen appears to yield the best results.

Keywords: Intravascular B-cell lymphoma; Lymphedema

Elsevier

|  Add to del.icio.us |  digg this