Clinical heterogeneity and diagnostic challenges in poems syndrome and variant: A case series from Southeast Asia

Abstract

POEMS syndrome is a rare paraneoplastic disorder associated with an underlying plasma cell dyscrasia presenting with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. Diagnosis can be challenging especially among those with atypical presentation. The diagnosis of POEMS was based on published criteria. We reviewed the clinical, laboratory and imaging characteristics of 4 patients in Kuala Lumpur Hospital from year 2012-2018. Clinical spectrum and disease variant were determined. Three had POEMS syndrome and 1 had Castleman disease variant. Mean age of onset was 40 (SD 11.5), with male predominant (75%). All had refractory chronic demyelinating polyneuropathy to standard CIDP treatment as common presentation. Paraprotein IgA lambda from monoclonal plasma cell-proliferative disorder were found in 2 patients. Sclerotic bone lesion (100%) was the most common major criteria fulfilled. Organomegaly (75%) was the most common minor criteria fulfilled, followed by extravascular volume overload (oedema, 50%, pleural effusion 25%), papilledema (25%), thrombocytosis (25%) and acrocyanosis (25%). Finger clubbing was seen in 50% of the patients. The only surviving patient with Castleman variant had improvement of neuropathy following IV rituximab and was in remission following autologous bone marrow transplant. Rapidly progressive disease was observed in one patient, with clinical deterioration to completely bedbound within a year. In conclusion, POEMS syndrome is a rare disorder and should be suspected in atypical cases of CIDP particularly when there is treatment resistance. Extensive investigation is often needed to meet diagnostic criteria for POEMS and rapid referral to haematology team for definite treatment is warranted.