Criteria for the diagnosis of POEMS syndrome
. | Symptom/Sign . |
|---|---|
| Major criteria | |
| Polyneuropathy | |
| Monoclonal plasma cell proliferative 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 B12 values | |
| Diarrhea |
. | Symptom/Sign . |
|---|---|
| Major criteria | |
| Polyneuropathy | |
| Monoclonal plasma cell proliferative 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 B12 values | |
| Diarrhea |
The research presented in this table was originally published in Dispenzieri A, Kyle RA, Lacy MQ, et al. POEMS syndrome: definitions and long-term outcome. Blood. 2003;101:2496-2506.
Please note that 2 major criteria and at least one minor criterion are required for diagnosis.
Osteosclerotic lesion or Castleman disease is almost always present.
Because of the high prevalence of diabetes mellitus and thyroid abnormalities, this diagnosis alone is not sufficient to meet this minor criterion.
Though the known and possible associations are not required to make a POEMS diagnosis, they lend weight to the diagnosis of the syndrome.