Criteria for the diagnosis of POEMS syndrome*
Major criteria | Polyneuropathy |
Monoclonal plasmaproliferative disorder | |
Minor criteria | Sclerotic bone lesions† |
Castleman disease† | |
Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy) | |
Edema (edema, pleural effusion, or ascites) | |
Endocrinopathy (adrenal, thyroid,‡ pituitary, gonadal, parathyroid, pancreatic‡) | |
Skin changes (hyperpigmentation, hypertrichosis, plethora, hemangiomata, white nails) | |
Papilledema | |
Known associations | Clubbing |
Weight loss | |
Thrombocytosis | |
Polycythemia | |
Hyperhidrosis | |
Possible associations | Pulmonary hypertension |
Restrictive lung disease | |
Thrombotic diatheses | |
Arthralgias | |
Cardiomyopathy (systolic dysfunction) | |
Fever | |
Low vitamin B12values | |
Diarrhea |
Major criteria | Polyneuropathy |
Monoclonal plasmaproliferative disorder | |
Minor criteria | Sclerotic bone lesions† |
Castleman disease† | |
Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy) | |
Edema (edema, pleural effusion, or ascites) | |
Endocrinopathy (adrenal, thyroid,‡ pituitary, gonadal, parathyroid, pancreatic‡) | |
Skin changes (hyperpigmentation, hypertrichosis, plethora, hemangiomata, white nails) | |
Papilledema | |
Known associations | Clubbing |
Weight loss | |
Thrombocytosis | |
Polycythemia | |
Hyperhidrosis | |
Possible associations | Pulmonary hypertension |
Restrictive lung disease | |
Thrombotic diatheses | |
Arthralgias | |
Cardiomyopathy (systolic dysfunction) | |
Fever | |
Low vitamin B12values | |
Diarrhea |
POEMS indicates polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes.
Two major criteria and at least 1 minor criterion are required for diagnosis.
Osteosclerotic lesion or Castleman disease is usually present.
Because of the high prevalence of diabetes mellitus and thyroid abnormalities, this diagnosis alone is not sufficient to meet this minor criterion.