IgD-λ POEMS syndrome presenting with edema, pulmonary hypertension, and renal impairment: A case report.
Jie Feng, Junle Zhao, Chao Song et al.
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Case in brief
A 38-year-old woman presented with years of intermittent leg edema, elevated creatinine, and acute worsening, followed by precapillary pulmonary hypertension, polyserous effusions, splenomegaly, lymphadenopathy, anemia, and thrombocytosis. Routine serum and urine protein studies, including immunofixation, were negative. Evaluation identified peripheral neuropathy, elevated VEGF, and a λ-restricted clonal plasma-cell population; extended immunofixation detected a trace IgD monoclonal component, establishing IgD-λ POEMS syndrome. Renal biopsy showed glomerular microangiopathy-like lesions and acute tubular injury. After dexamethasone acetate, lenalidomide, and autologous stem-cell transplantation, edema resolved, pulmonary hypertension became mild, creatinine decreased to 60 μmol/L, and VEGF normalized.
What made this case unusual
The low-level IgD-λ component was detectable only with extended immunofixation using anti-IgD and anti-IgE antisera, despite negative routine protein studies, in a presentation featuring pulmonary hypertension and renal impairment.
Diagnostic / clinical pearl
In unexplained multisystem disease with edema or effusions, pulmonary hypertension, and renal impairment, negative routine M-protein testing does not exclude POEMS; VEGF, marrow evaluation, and extended immunofixation may uncover an occult clone.