La Revue de médecine interne
-
Case Reports
[Gamma heavy chain disease associated with aggressive B lymphoma in the context of myelodysplastic syndrome].
Heavy chain disease is a rare entity characterized by the production of incomplete immunoglobulin heavy chain without associated light chain. It is a B-cell lymphoproliferation, categorized according to the immunoglobulin involved. It is often associated with lymphomas but also with autoimmune diseases. ⋯ This is the first case of diffuse large B-cell lymphoma associated gamma heavy chain disease described in the context of myelodysplastic syndrome.
-
POEMS syndrome is a rare form of B-cell dyscrasia with multiple clinical signs including the acronym for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. It is a paraneoplastic syndrome due to an underlying plasma cell disorder belonging to the monoclonal gammopathies of clinical significance (MGCS). The major criteria for this syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor (VEGF), and the presence of Castleman's disease. ⋯ Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3 to 6 months after completing irradiation therapy should receive antiplasma cell systemic therapy, the most effective being high dose chemotherapy with autologous stem cell transplantation. Lenalidomide seems to have a high efficacy with manageable toxicity. Thalidomide and proteasome inhibitors like bortezomib are also effective, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy.