Table 2

Proposed and existing diagnostic criteria of POEMS syndrome

 Proposed criteria*Dispenzieri,4 5
Kuwabara et al 6†		Dispenzieri et al 7‡
Major criteria
 1.Polyneuropathy (typically demyelinating)§1.Polyneuropathy (typically demyelinating)§1.Polyneuropathy§
 2.Monoclonal plasma cell proliferative disorder§2.Monoclonal plasma cell proliferative disorder§2.Monoclonal plasmaproliferative disorder§
 3.Vascular endothelial growth factor elevation§3.Castleman disease¶
 4.Sclerotic bone lesions
 5.Vascular endothelial growth factor elevation
Minor criteria
 4.Extravascular volume overload6.Organomegaly3.Sclerotic bone lesions
 5.Skin changes7.Extravascular volume overload4.Castleman disease
 6.Organomegaly8.Endocrinopathy5.Organomegaly
 7.Sclerotic bone lesions9.Skin changes6.Oedema
 10.Papilloedema7.Endocrinopathy
 11.Thrombocytosis/polycythaemia8.Skin changes
 9.Papilloedema

  • *All of the three major criteria and at least two of the four minor criteria are required for diagnosis.

  • †Both of the mandatory criteria, one of the three other major criteria and at least one of the six minor criteria are required for diagnosis.

  • ‡Both of the mandatory criteria and at least one of the seven minor criteria are required for diagnosis.

  • §Mandatory criteria.

  • ¶There is a Castleman disease variant of POEMS syndrome that occurs without evidence of a clonal plasma cell disorder that is not accounted for in this table. This entity should be considered separately.

  • POEMS, polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes.