Assuntos
Hiperplasia do Linfonodo Gigante/diagnóstico , Neoplasias Cerebelares/diagnóstico , Cordoma/diagnóstico , Meningioma/diagnóstico , Acuidade Visual , Adulto , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/líquido cefalorraquidiano , Hiperplasia do Linfonodo Gigante/etiologia , Neoplasias Cerebelares/complicações , Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/cirurgia , Proteínas do Líquido Cefalorraquidiano , Cordoma/complicações , Cordoma/diagnóstico por imagem , Cordoma/cirurgia , Extremidades/patologia , Feminino , Febre/patologia , Cefaleia/patologia , Humanos , Hipoalbuminemia , Interleucina-6/sangue , Imageamento por Ressonância Magnética , Meningioma/complicações , Meningioma/diagnóstico por imagem , Meningioma/cirurgia , Bandas Oligoclonais/líquido cefalorraquidiano , Parestesia/patologia , Resultado do TratamentoRESUMO
We report six patients affected by POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes), a peculiar multiorgan disease frequently associated with osteosclerotic myeloma or other plasma cell disorders. Sensorimotor polyneuropathy was associated with multisystem involvement in all of the patients, with osteosclerotic myeloma in 2 cases, monoclonal gammopathy of undetermined significance in 2 cases and Castleman's disease in the final two. In all of the patients, sural nerve biopsy findings were consistent with a mixed, axonal and demyelinating neuropathy. Increased levels of Interleukin-6 were found in two cases, but the pathogenesis of the disease is far from established.
Assuntos
Síndrome POEMS/fisiopatologia , Idoso , Medula Óssea/patologia , Hiperplasia do Linfonodo Gigante/líquido cefalorraquidiano , Hiperplasia do Linfonodo Gigante/patologia , Eletrofisiologia , Hormônios/sangue , Humanos , Interleucina-6/líquido cefalorraquidiano , Linfonodos/patologia , Masculino , Pessoa de Meia-Idade , Neurônios Motores/fisiologia , Condução Nervosa/fisiologia , Osteosclerose/patologia , Síndrome POEMS/líquido cefalorraquidiano , Síndrome POEMS/patologia , Paraproteinemias/patologia , Prognóstico , Nervo Sural/patologia , Fator de Necrose Tumoral alfa/líquido cefalorraquidianoRESUMO
POEMS syndrome is a rare multisystem affliction known for its signs, from which it also takes its acronym name "peripheral neuropathy, organomegaly, endocrinopathy, monoclonal (M) protein, and skin lesions." Our study chronicles the course of this syndrome in a young woman with Castleman's disease (angiofollicular lymph node hyperplasia). Cerebrospinal fluid (CSF) and serum interleukin-6 (IL-6) levels were abnormally elevated at various times during a 9-month period. The authors conclude that the plasma cell dyscrasia associated with the POEMS syndrome of this patient was Castleman's disease. Elevation of serum IL-6 levels might contribute to the pathogenesis of the POEMS syndrome.
Assuntos
Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/líquido cefalorraquidiano , Interleucina-6/análise , Síndrome POEMS/sangue , Síndrome POEMS/líquido cefalorraquidiano , Adulto , Axila , Biópsia , Hiperplasia do Linfonodo Gigante/complicações , Hiperplasia do Linfonodo Gigante/fisiopatologia , Feminino , Humanos , Interleucina-6/sangue , Interleucina-6/líquido cefalorraquidiano , Linfonodos/patologia , Condução Nervosa , Síndrome POEMS/complicações , Síndrome POEMS/fisiopatologia , Nervo Frênico/fisiopatologiaRESUMO
A case of Castleman's disease of the plasma-cell type is reported in which central nervous system (CNS) involvement was diagnosed by cerebrospinal fluid (CSF) cytology. The patient had multicentric disease with constitutional symptoms, immunologic abnormalities and peripheral blood cytopenias requiring cytotoxic agents and steroids for treatment. CNS symptoms and diagnostic cytologic findings in CSF occurred in the absence of morphologic lesions demonstrable by computed tomography or magnetic resonance imaging of the brain.