Özet:

POEMS syndrome defines a rare multisystem syndrome due to the paraneoplastic manifestation of plasma cell dyscrasia. The diagnosis is based on the clinic and the laboratory: this acronym includes part of all the current criteria on this disease: Polyradiculoneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disease (PCD) and Skin changes. Peripheral neuropathy is often the revealing symptom of the disease. Bone involvement is present in 54 to 97% and is typically osteosclerotic. POEMS syndrome with plasmacytoma lytic bone lesion remain exceptional. In the case of patients with less than three solitary bone plasmacytomas, radiotherapy is recommended as a therapeutic approach with variable efficacy. Relapses remain frequent requiring systemic treatment. The management of the pandemic had indirect effects on serious diseases in fact, fear of the crisis caused by the epidemic is pushing a large number of patients to delay their request for care. In addition to chronic patient monitoring, the practitioners primary concern is to detect emergency situations. We report the case of a 45-year-old right-handed patient with a progressive symmetrical ascending four-limb sensory motor deficit over the past five months, suggesting polyradiculoneuropathy, associated with rib pain from the 7th to the 9th right ribs. The assessment confirms demyelinating and axonal polyradiculoneuropathy and demonstrates the presence of a monoclonal IgG λ gammopathy, in connection with a solitary osteolytic right costal plasmacytoma of the 7th rib, without organomegaly on thoraco-abdomino-pelvic CT scan. We suspected the diagnosis of POEMS syndrome which was confirmed by elevated serum VEGF dosage. He is receiving corticosteroid therapy with localized radiotherapy with good clinical outcome and biological stabilization. However, the patient had presented during confinement for Covid-19 a worsening of the progressive symptomatology evolving for 6 months. He presented to the emergency room with a picture of flaccid quadriplegia and fatal respiratory distress.

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