ASCIA‐P75: ELEVATED VASCULAR ENDOTHELIAL GROWTH FACTOR (VEGF) PLAYS AN IMPORTANT ROLE IN THE DIAGNOSIS OF POEMS SYNDROME

Abstract

POEMS syndrome is a rare paraneoplastic syndrome that is caused by an underlying plasma cell disorder. It is characterised by polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. The diagnosis is challenging due to the multifaceted nature of its manifestations. VEGF is one of the major diagnostic criteria of the syndrome. A 40 year-old man developed severe fatigue, footdrop and ascending weakness. IVIG was initiated for the presumptive diagnosis of CIDP with little effect. Corticosteroids, plasmapheresis, mycophenolate and rituximab did not halt the progression of his symptoms. Within one year, there was a significant deterioration in his fine motor skills and ability to walk. Significant findings on investigations included an IgA lambda paraprotein on serum IFE, a trace of paraprotein in 24-hour urine collection, sclerotic bone lesions, decreased DLCO on lung function test, incidental pulmonary emboli on CTPA, mild splenomegaly, prolactinaemia and low testosterone with raised FSH and LH indicative of primary hypogonadism. A VEGF level was obtained from a research laboratory showing significant elevation at 3292 pg/ml (normal control 778 pg/ml). The VEGF level was instrumental in reaching a unifying diagnosis of POEMS syndrome in this patient who was then able to undertake definitive treatment of chemotherapy with autologous stem cell transplant for curative intent. This case illustrates the utility of VEGF in diagnosing a rare yet devastating syndrome that often presents as a diagnostic dilemma with multisystem manifestations. VEGF levels correlate with disease activity rendering it a useful assay for monitoring the patient's response to therapy. VEGF is only available in research settings at present. Increased awareness of its utility will not only optimise the investigation and management of POEMS but also assist in the advocacy of adding the test to the MBS schedule for its use in clinical practice.