Necrobiotic Xanthogranuloma With Non- Progressive Hematological Dyscrasia, A Case Report

Abstract

Necrobiotic Xanthogranuloma (NXG) is a rare systemic and progressive histiocytosis of unknownaetiologyin which 80% of the cases is considered a marker of paraproteinemia, a monoclonal gammopathy IgG sometimes related with Multiple Myeloma (MM), it has also been associated with other progressive and non-progressive hematologic dyscrasias. Although its location in the periorbital area is distinctive, its course can be systemic and generalized. In the beginning, the diagnosis is difficult and its clinical-pathological correlation becomes necessary. Its prognosis is generally good, but currently there is no standardized or effective treatment;surgery is an option yet with high tendency for recurrence. We present aXNG clinical case associated with autoimmune idiopathic thrombocytopenia, which has remained stable for more than 15 years, although previous reports have shown this association more with adult orbital xanthogranulomathan NXG itself, the clinical significance is yet to be knownaccording to this spectral, infrequent and enigmatic condition.