POEMS syndrome is a rare paraneoplastic disorder associated with underlying plasma cell dyscrasias for which a dearth of literature exists on optimal therapy.  Prior cases have utilized stem-cell transplant or systemic agents usually developed for treatment of multiple myeloma.  In addition, patients may receive treatments based on incorrect diagnoses before POEMS is diagnosed. 

A 41-year-old male was diagnosed with POEMS after 6 months of progressive bilateral ascending sensorimotor deficits in the setting of paraproteinemia, hepatosplenomegaly, endocrinopathies, and skin changes.  Studies showed an elevated VEG-F level and plasma cell dyscrasias within the T8 vertebrae and hip bone marrow aspirate.  A poor candidate for stem-cell transplant, he was started on treatment of 25 daily irradiation therapy sessions to the T8 lesion and Daratumumab 16 mg/kg weekly.  Before treatment for POEMS, he received eculizumab and tocilizumab.

Despite 4 weeks of treatment, the patient demonstrated disease progression with no symptomatic improvement. Exam was notable for development of hemiplegia of the legs, worsening paresis of the arms, and severe anasarca. He progressed to renal failure requiring dialysis. Atypically, the patient also developed hepatic dysfunction seen as hyperbilirubinemia and hyperammonemia, as well as refractory thrombocytopenia and normocytic anemia.  He passed away 4 weeks after starting therapy.

There is uncertainty regarding the choice of treatment for patients with POEMS syndrome though regimens target the underlying plasma cell dyscrasia.  Our use of daratumumab contrasts its success as salvage-therapy in a prior case.  In addition, our patient developed liver dysfunction and abnormal blood counts either due to progression of POEMS or daratumumab usage, neither of which are reported in literature.  Treatment with eculizumab and tocilizumab may also have contributed to his comorbidities.