Rare reports of pulmonary manifestations of poems syndrome such as pulmonary hypertension exist. Poems syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Poems syndrome, monoclonal gammopathy, thrombo cytosis. Sindrome poems informazioni su cause, sintomi, diagnosi e trattamento disponibili su manuali msd, versione per i pazienti. The physiopathology of poems is shared by a number of entities including paraneoplastic syndromes and even some diseases with selfimmune characteristics such as those related to igg4 and infectious processes, but above all with blood diseases such as the tafro syndrome, a variant of castlemans disease, the diagnostic criteria of which. This condition has no cure but society can play its role by giving the affected individual more understanding and acceptance. Pulmonary manifestations in patients with poems syndrome. Plasmacell dyscrasia with polyneuropathy, organomegaly. Half of these patients 1428 had mild increases in thyrotropin levels 5 but poems syndrome is a unique clinical entity defined by the presence of a. Apropos of a case abstract poems syndrome is a rare clinical entity with multisystem condition, resulting from an alteration of the plasma cells. Poems syndrome also termed osteosclerotic myeloma, crowfukase syndrome, takatsuki disease, or pep syndrome is a rare paraneoplastic syndrome caused by a clone of aberrant plasma cells. To our knowledge, only one other case of phrenic neuropathy in. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder pcd, sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of castleman disease.
In 1956, crow reported two patients with osteosclerotic plasmocytoma, peripheral neuropathy, cutaneous pigmentation, leukonychia. Thyroid axis elevated thyrotropin concentrations were noted in 28 58% of 48 patients 17 men and 11 women. Phrenic nerve paresis is an unusual complication of poems polyneuropathy, organomegaly, endocrinopathy, mprotein spike and skin changes syndrome. Find, read and cite all the research you need on researchgate. In this report, we describe a case of poems syndrome in which a 56yearold woman presented with dyspnea and ventilatory failure due to bilateral phrenic nerve paralysis. Sindrome poems disturbi ormonali e metabolici manuale msd.
Caso clinico ajbp, sexo masculino, 41 anos ingreso. Poems syndrome results from a rare proliferative monoclonal disease of systemic plasmacyte attack with indolent course. Discover free books by george orwell, who are publishing novels, thriller, poems. To our knowledge, only one other case of phrenic neuropathy in poems. Being a multisystemic disorder, poems syndrome has different clinical features. Mass reproduce only with permission from mayo clinic proceedings. Down syndrome quotes for awareness and acceptance enkiquotes. Poems syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm.