POEMS syndrome

POEMS syndrome is a constellation of findings, best thought of as a paraneoplastic syndrome due to clonal plasma cells:

• Polyneuropathy.
• Organomegaly.
• Endocrinopathy.
• M-protein.
• Skin changes.

Diagnostic criteria

Requires both mandatory criteria and at least one each from major and minor criteria:

Mandatory:

1. Polyneuropathy (typically demyelinating).
2. Monoclonal plasma cell proliferation (usually lambda).

Major:

1. Castleman disease
2. Sclerotic bone lesions.
3. Vascular endothelial growth factor elevation.

Minor:

1. Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy).
2. Extravascular volume overload.
3. Endocrinopathy.
4. Skin changes.
5. Papilloedema.
6. Thrombocytosis/polycythemia.

Pathology

• The pathologist may come across an undiagnosed case in the form of a glomeruloid hemangioma; however, glomeruloid hemangiomas are not always associated with the POEMS syndrome.
• POEMS syndrome typically cause osteosclerotic bone deposits (so-called osteosclerotic myeloma), in contrast to the lytic lesions seen in more conventional plasma cell myeloma.^{[citation needed]}

See also

• POEM - a treatment for achalasia.