La Revue de médecine interne
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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.
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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.
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Review Case Reports
[Cytomegalovirus infection in systemic lupus erythematosus patients. A series of 12 case reports and literature review].
Patients with systemic lupus erythematosus (SLE) are at risk of cytomegalovirus (CMV) infection, due to the disease itself or to drug-induced immunosuppression. Also, active CMV infection may trigger or worsen SLE flare-up. ⋯ Among patients with SLE, CMV infection affected more frequently those treated with immunosuppressive drugs, but treatment-free patients were sometimes severely affected. CMV infection was associated with an increased incidence of SLE flare-up and infectious complications. Our results suggest that early anti-viral chemotherapy may be beneficial in these patients.
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Parkinson's disease is the second most common neurodegenerative disease after Alzheimer's disease. The pathophysiology of Parkinson's disease is complex and imperfectly known. Primum movens is abnormal intra-neuronal accumulation of the protein α-synuclein, leading to metabolic disturbances and neurodegeneration. ⋯ Furthermore, non-motor symptoms have a poorer impact on quality of life than motor signs themselves. Therefore, understanding, recognition and management of non-motor symptoms are crucial in management of parkinsonian patient. In this paper, we offer an update on the main non-motor symptoms of Parkinson's disease, from their pathophysiology to their screening, ending with their management.