POEMS síndrome is an uncommon multisystemic paraneoplastic disorder characterized by the presence of a predominantly motor polyneuropathy associated with other manifestations like organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. We report a case of a 36 years old female with diagnostic criteria for this disorder, and a particular double monoclonal pattern IgG and IgA type., demonstrated by immunofixation.
POEMS syndrome
Síndrome de POEMS: Múltiples manifestaciones clínicas durante su larga evolución.
Being a multisystemic disorder, POEMS syndrome has different clinical features.and comprises multiple organs. The referred acronym: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein M and skin changes, results insufficient when this syndrome is described, because of the constellation of multiple signs and symptoms. Neurologically a chronic demyelinating peripheral neuropathy is the common clinical presentation, which is associated with an osteosclerotic myeloma in 50% of cases. Involvement of central nervous system includes papilledema and intracraneal hypertension, less frequently. We report a patient with POEMS syndrome with 11 years follow-up, who has recently developed intracraneal hypertension features. We discuss clinical findings and review literature.
