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Multicentric Angiofollicular Lymph Node Hyperpl with Neuropathy, Pseudotumor Dysprot & Thrombocytosis
Ann Int Med 113:362-367, Feigert,J.M.,et al, 1990
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Article Abstract
Four women with multicentric angiofollicular lymph node hyperplasia had a distinct clinical syndrome characterized by peripheral neuropathy, pseudotumor cerebri,IgA dysproteinemia,and thrombocytosis.The nodes displayed typical morphologic changes of the plasma cell variant of multicentric angiofollicular lymph node hyperplasia.The pathologic changes are morphologically distinct from angioimmunoblastic lymphadenopathy with dysproteinemia although clinical similarities do exist.In these four cases,the lymphadenopathy was usually bulky and multicentric.There was frequent splenic involvement.The neuropathies were severe and disabling. Clinical courses have been variable with some responses to therapy with steroids and alkylating agents.No neoplastic transformations have occurred.Multicentric angiofollicular lymph node hyperplasia may represent a reactive lesion in which the antigenic stimulus is unknown but results in follicular hyperplasia,angiogenesis,and the systemic manifestations of hyperimmune stimulation.We believe this clinical syndrome may represent a distinct variant of multicentric angiofollicular lymph node hyperplasia, and it requires close observation for neoplastic transformation and other complications of its multisystem nature.
 
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Castleman's disease
dysproteinemia
intracranial hypertension,benign
lymphadenopathy
neuropathy
neuropathy,peripheral
thrombocytosis

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