CASTLEMAN DISEASE VARIANT OF POEMS SYNDROME. A CASE REPORT
SÍNDROME POEMS ASOCIADO A ENFERMEDAD DE CASTLEMAN. UN REPORTE DE CASO
DOI:
https://doi.org/10.25176/RFMH.v19i4.2145Keywords:
POEMS syndrome, Castleman's disease, PlasmapheresisAbstract
Introduction: POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) syndrome is a rare multisystem disorder. Castleman disease is an atypical lymphoproliferative disorder of unknown cause, which can be associated to POEMS.
Clinical Case: This is the case of a 37-year-old patient with 2-year history of numbness and weakness in lower limbs associated to multiple adenopathy, skin hyperpigmentation and hypertrichosis. Electromyography showed active chronic sensory-motor polyneuropathy of axonal type; and serum electrophoresis showed a monoclonal band of A Immunoglobulin lambda type. Submaxillary lymph node biopsy was consistent with Castleman Disease.
Conclusion: This report highlights the fact that Castleman Disease is frequent among patients with POEMS syndrome, constituting a significant variant, and should always be ruled out. Furthermore, plasmapheresis could be a useful alternative among patients with severe neurological symptoms.
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