Introduction: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) is a rare multisystem paraneoplastic disorder resulting from plasma cell dyscrasia. Pulmonary hypertension (PH) is a complication of POEMS. Here, we present a case involving a young woman diagnosed with POEMS syndrome complicated by new-onset pulmonary hypertension, and pericardial effusion. Description: A female in her 20s with celiac disease and Hashimoto’s thyroiditis presented in late 2024 with progressive demyelinating peripheral neuropathy, initially misdiagnosed as Guillain–Barré syndrome. She received IVIG without improvement. By early 2025, she developed bilateral lower extremity edema and severe generalized anasarca, prompting hospital admission. Vitals showed tachycardia (130 bpm), BP (90/60 mmHg). Exam revealed JVD, 4+ pitting edema, ascites, and symmetric lower limb weakness with hyporeflexia. BNP was >1000 pg/mL, troponin normal, TSH elevated. ECG showed sinus tachycardia. Echocardiogram demonstrated moderate pericardial effusion, normal LVEF, reduced RV function, and estimated PASP of ~60 mmHg without evidence of tamponade. Diuresis with IV furosemide led to hypotension (MAP 50 mmHg), requiring ICU transfer. Arterial line demonstrated significant pulsus paradoxus. RHC showed precapillary pulmonary hypertension without diastolic pressure equalization. During hospitalization, serum protein electrophoresis revealed a monoclonal IgA lambda paraprotein. The combination of monoclonal gammopathy, endocrinopathy, and progressive polyneuropathy raised concern for POEMS syndrome. Serum VEGF was markedly elevated. Bone marrow biopsy showed ~5% lambda light-chain–restricted clonal plasma cells, confirming POEMS. Her anasarca became severe, she developed abdominal compartment syndrome, she developed ventricular tachycardia and died despite resuscitation. Discussion: Pulmonary hypertension in POEMS may occur in up to 27% of cases due to VEGF-mediated endothelial dysfunction and vascular remodeling. Treating the plasma cell disorder can reverse PH. Pulsus paradoxus is not specific to tamponade and may reflect RV dysfunction or PH. In cases with overlapping features, RHC is essential to distinguish between tamponade and precapillary PH, as echocardiography may not show classic signs.
Krishtopaytis et al. (Sun,) studied this question.