POEMS Syndrome - 30/01/25
, Virginie Pascal 2, 3, Laurent Magy 4, Murielle Roussel 1, 2Abstract |
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, Skin changes) is a syndrome that involves a monoclonal B-cell proliferation, most often plasmacytic, and a variable number of manifestations listed or not in the acronym. These manifestations include sclerotic bone lesions, plasmacytic Castleman disease, papillary edema, peripheral edema, ascites, thrombocytosis and/or polycythemia, venous and/or arterial thrombosis, and renal, pulmonary, and cardiac impairments [1]. Diagnosis is often delayed due to the rarity of this entity and its clinical polymorphism, which can mimic other neurological disorders. First-line treatment for patients without bone marrow involvement and with a limited number of bone lesions is radiation. Patients with diffuse bone lesions or bone marrow involvement should receive systemic treatment, ideally intensive treatment with autologous stem cell transplantation (ASCT) when possible. Lenalidomide and bortezomib (Bortezomib) appear to be very promising, showing very rapid efficacy on neuropathy. Early initiation of treatment, before the development of severe neurological damage, along with supportive care, especially physiotherapy, is crucial for optimal neurological recovery.
Le texte complet de cet article est disponible en PDF.Keywords : POEMS syndrome, MGCS, Castleman's disease, VEGF, Radiation, Autologous stem cell transplantation, Treatment
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Vol 54 - N° 1
Article 104270- mars 2025 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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