Table 4.

Adapted Response Criteria in POEMS Syndrome^1,55

Clinical response
Based on patient and physician qualitative reports of constitutional symptoms and systemic features
• Clinical improvement (Ci)
• Clinical progression (Cp)
• Mixed clinical response (Cm)
• Clinical stability (Cs)
Neuropathy response
Defined by clinical examination, neurophysiology, i-RODS (POEMS-RODS in future), modified Rankin Score56 and ONLS.57 Progression defined as worsening of functional ability by an increase in i-RODS of>4 or ONLS by ≥1
Hematological response
• Complete response (CRH)—Negative bone marrow (if originally involved) and negative immunofixation of serum and urine.
• Very good partial response (VGPRH)—90% reduction in the M-protein or immunofixation positive only, provided M-protein ≥5 g/L at baseline.
• Partial response (PRH)—90% reduction in M-protein or immunofixation positive, provided baseline M-protein ≥10g/L.
• No response—Less than PRH.
• Progression—reemergence of M-protein in serum and/or urine or an increase of ≥25% from posttreatment nadir, but the M-protein must be >5g/L.
VEGF response
• Complete response (CRVEGF)—Normalization of VEGF (<771 pg/mL)
• Partial response (PRVEGF)—Decrease of ≥50% (baseline must be ≥2000 pg/mL)
• No response (NRVEGF)—Less than a PRVEGF
• Progression—Persistent (≥2 recordings) VEGF elevation ≥771pg/ml from a previously normal result or a persistent rise in VEGF of >50% from posttreatment nadir (if <CRVEGF achieved)
Radiological response
• Complete radiologic response (CRR)—Resolution of FDG-avidity
• Partial radiologic response (PRR)—FDG-avidity improved by ≥50%
• No radiologic response—Less than 50% reduction in FDG-avidity
• Progression—30% increase in sum of SUVmax from lowest level, but must be at least 4 SUVmax OR the appearance of a new FDG-avid lesion

FDG = fluorodeoxyglucose; ONLS = Overall Neuropathy Limitation Score; POEMS = Polyneuropathy Organomegaly, Endocrinopathy, M-protein and Skin changes; VEGF = vascular endothelial growth factor.