Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Associated With Castleman Disease: A Common Misdiagnosis

Abstract

Castleman disease is a rare lymphoproliferative disorder. Co-presentation with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS syndrome) has been documented in 11-30% of Castleman disease cases. POEMS syndrome is a rare paraneoplastic disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. Not all features are required to make the diagnosis. We report a case of a woman who presented with a 1-year history of a left-side supraclavicular swelling associated with constitutional symptoms and symmetrical paresthesia of the lower limbs. In addition, she had skin hyperpigmentation, multiple supra and infra-diaphragmatic lymphadenopathies, hepatosplenomegaly and osteosclerotic lesions. Serum immunofixation was positive for immunoglobulin G-kappa gammopathy. A lymph node excisional biopsy was compatible with Castleman disease. The diagnosis of POEMS syndrome associated with Castleman disease was made. Our patient started treatment with a combination of bortezomib, cyclophosphamide, and dexamethasone with clinical and analytical improvement. Current treatment of POEMS syndrome associated with Castleman disease is focused on the management of POEMS syndrome. Early diagnosis requires a high index of suspicion and is crucial to reduce morbidity and mortality. This case report aims to raise awareness about this rare entity.

POEMS syndrome - a diagnostic dilemma with challenging presentation

“…Based on previous literature and as seen with our patient, diagnosis can be achieved approximately one to two years after initial symptom presentation. 4 , 5 Common misdiagnosis include CIDP, Monoclonal Gammopathy of Undetermined Significance (MGUS) or multiple myeloma. 6 …”

Enfermedad de castleman multicéntrica. Reporte de caso.

“…7-N° 3, 2023, pp. 3379-3397 Journal Scientific MQRInvestigar 3385 NCDHHV8+/HIV+ ha aumentado en los últimos años (9). La enfermedad de Castleman es una enfermedad rara.…”

“…A nivel bioquímico suele cursar con alteraciones como la trombocitopenia, leucocitosis, hipoalbuminemia, anemia, VSG, IL-6 y fibrinógeno elevado (8). Entre la forma grave de presentación de la enfermedad se describe el síndrome de POEMS caracterizado por falla multiorgánica, amiloidosis, linfoma y pancitopenia (9).…”