Morning Report 3/15/18 Kathryn Rimmer POEMS vs CIDP Morning Report 3/15/18 Kathryn Rimmer

POEMS Syndrome Polyneuropathy Organomegaly Endocrinopathy M-protein Skin changes = hyperpigmentation, skin thickening, excessive hair growth Extremely rare multisystem disorder

Diagnostic Criteria Required Criterion Polyneuropathy M-protein (usually lambda) Major Criterion (>1) Sclerotic bone lesion Elevated VEGF Castleman disease Minor Criterion (>1) Organomegaly liver, spleen, lymph node Endocrinopathy Skin change Edema Polycythemia Thrombocytosis Papilledema

Clinical Phenotype Similar to CIDP Symmetric length-dependent sensorimotor polyneuropathy Motor > sensory symptoms Progressive course Absent reflexes CSF with albuminocytologic dissociation NCS/EMG with features of demyelination and axonal loss; patterns may resemble CIDP

Distinguishing Features of POEMS Thrombocytosis 54% of POEMS patients, 1.5% of CIDP patients Elevated VEGF Sclerotic lesion(s) on skeletal survey or PET/CT Associated with higher risk of thrombotic events NCS/EMG Greater attenuation of motor and sensory responses in legs vs arms Modest distal latency prolongation CV slowing more prominent in intermediate nerve segments Evidence of axonal loss in legs Conduction block rarely seen

Treatment XRT Chemotherapy Autologous stem cell transplant