To present a case series of two patients with POEMS syndrome and their varied clinical presentations, highlighting the importance of considering it as a differential of chronic demyelinating neuropathies.
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes) is a rare paraneoplastic disorder that is associated with plasma cell dyscrasias. The pathophysiology revolves around elevated Vascular Endothelial Growth Factor (VEGF) and other cytokines, which correlates with the clinical severity and disease activity. Timely diagnosis is crucial to prevent irreversible disability.
Case 1: A 36-years old male presented with insidious onset progressive symmetric sensorimotor flaccid paraparesis, associated with weight loss. Examination showed hyperpigmentation, hepatosplenomegaly, generalized lymphadenopathy and papilledema. Nerve conduction study (NCS) showed demyelinating sensorimotor polyneuropathy. In view of multisystem involvement associated with weight loss, PET-CT was done which showed osteosclerotic lesions. Further workup in the form of serum electrophoresis revealed IgG lambda monoclonal protein, with bone marrow biopsy showing 40% plasma cells with abnormal monoclonal protein. VEGF levels were found to be elevated, thus fulfilling the diagnostic criteria for POEMS.
Case 2: 51-years old male presented with 7-years history of sensorimotor flaccid quadriparesis. NCS showed demyelinating polyneuropathy, and the patient was initially worked up as CIDP and started on IVIG, but with no adequate response. Detailed evaluation showed associated endocrinopathy (hypothyroidism, hypogonadism and hypertrichosis) with hepatosplenomegaly. POEMS syndrome was confirmed by serum immunofixation and bone marrow biopsy showing IgG lambda light chains.
Both patients were started on bortezomib based chemotherapy, and one of them underwent autologous stem cell transplant, leading to symptomatic improvement.
POEMS syndrome is an important differential to be ruled out in cases of chronic demyelinating polyneuropathy. Detailed evaluation, and early initiation of chemotherapy can lead to significant improvement in disease outcome.