Table 1 Clinical features of POEMS syndrome
CaseAge/SexOrganomegalyEndocrinopathyM-proteinSkin changeBone lesionVEGF level* (pg/ml)Cerebrospinal fluid protein (mg/dl)
143/WCM, HM, SM, LAIgAλHP, HTSclerotic131093
268/WCM, HM, SMIgAλHP, HTSclerotic229063
347/WHM, SMIgGλHP167038
447/WCM, HM, SM, LAHyIgAλHP, HT>200095
531/MHM, SMGy, ImIgGλHP, HTSclerotic, lytic5500130
654/WHM, LAIgGκHP, HTSclerotic756†107
748/MLAGyIgGλHPSclerotic183063
846/MHM, LAGyIgGλHPSclerotic>2000108
962/MSMGyIgAλHP, HTSclerotic>2000165
1058/MImIgAλHP, HTSclerotic>200053
1157/WCM, HM, SMHyIgAλHP, HTSclerotic>2000179
1228/WHM, SMIgGλHP, HTSclerotic, lytic1120238
1372/MHM, SMGuIgGλHPND124
1467/WCM, SM, LAHyIgGλHP>200097
1563/MHM, SM, LAHy, Gy, ImIgGλHP, HTND82
1645/MLAGu, Hy, ImIgAλHP, HTND196
1769/WNDIgAλHPND108
1860/MLAHyIgAλHP, HTND62
1963/MSMImHPSclerotic814†78
2045/MHMGu, GyHP, HT1280360
2185/MCM, HM, SM, LAGyIgAλHP500†57
2247/MHM, SMImIgGλHP, HTLytic128081
  • VEGF, vascular endothelial growth factor (normal<707 pg/ml for serum, <115 pg/ml for plasma); –, abscent; CM, cardiomegaly; Gu, glucose intorelance; Gy, gynecomastia; HM, hepatomegaly; HP, hyperpigmentation; HT, hypertrichosis; Hy, hypothyroidism; Im, impotence; LA, lymphadenopathy; ND, not determined; POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes; SM, splenomegaly.

  • *Serum VEGF levels were measured except in patients 6, 19 and 21.

  • †Plasma VEGF levels were measured.