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

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

3. 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

4. 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

5. 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

6. Treatment
XRT
Chemotherapy
Autologous stem cell transplant