Subject(s)
Castleman Disease/diagnosis , Cerebellar Neoplasms/diagnosis , Chordoma/diagnosis , Meningioma/diagnosis , Visual Acuity , Adult , Castleman Disease/blood , Castleman Disease/cerebrospinal fluid , Castleman Disease/etiology , Cerebellar Neoplasms/complications , Cerebellar Neoplasms/diagnostic imaging , Cerebellar Neoplasms/surgery , Cerebrospinal Fluid Proteins , Chordoma/complications , Chordoma/diagnostic imaging , Chordoma/surgery , Extremities/pathology , Female , Fever/pathology , Headache/pathology , Humans , Hypoalbuminemia , Interleukin-6/blood , Magnetic Resonance Imaging , Meningioma/complications , Meningioma/diagnostic imaging , Meningioma/surgery , Oligoclonal Bands/cerebrospinal fluid , Paresthesia/pathology , Treatment OutcomeABSTRACT
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.
Subject(s)
POEMS Syndrome/physiopathology , Aged , Bone Marrow/pathology , Castleman Disease/cerebrospinal fluid , Castleman Disease/pathology , Electrophysiology , Hormones/blood , Humans , Interleukin-6/cerebrospinal fluid , Lymph Nodes/pathology , Male , Middle Aged , Motor Neurons/physiology , Neural Conduction/physiology , Osteosclerosis/pathology , POEMS Syndrome/cerebrospinal fluid , POEMS Syndrome/pathology , Paraproteinemias/pathology , Prognosis , Sural Nerve/pathology , Tumor Necrosis Factor-alpha/cerebrospinal fluidABSTRACT
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.
Subject(s)
Castleman Disease/blood , Castleman Disease/cerebrospinal fluid , Interleukin-6/analysis , POEMS Syndrome/blood , POEMS Syndrome/cerebrospinal fluid , Adult , Axilla , Biopsy , Castleman Disease/complications , Castleman Disease/physiopathology , Female , Humans , Interleukin-6/blood , Interleukin-6/cerebrospinal fluid , Lymph Nodes/pathology , Neural Conduction , POEMS Syndrome/complications , POEMS Syndrome/physiopathology , Phrenic Nerve/physiopathologyABSTRACT
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.
