Criteria for the diagnosis of POEMS syndrome
| . | Criteria/other symptoms and signs . | Affected, %* . |
|---|---|---|
| Mandatory major criteria (both required) | 1. Polyradiculoneuropathy (typically demyelinating) | 100 |
| 2. Monoclonal plasma cell disorder (almost always λ) | 100† | |
| Other major criteria (1 required) | 3. Castleman disease‡ | 11-25 |
| 4. Sclerotic bone lesions | 27-97 | |
| 5. VEGF elevation§ | ||
| Minor criteria (1 required) | 6. Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy) | 45-85 |
| 7. Extravascular volume overload (edema, pleural effusion, or ascites) | 29-87 | |
| 8. Endocrinopathy (adrenal, thyroid,‖ pituitary, gonadal, parathyroid, pancreatic‖) | 67-84 | |
| 9. Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, white nails) | 68-89 | |
| 10. Papilledema | 29-64 | |
| 11. Thrombocytosis/polycythemia¶ | 54-88 | |
| Other symptoms and signs | Clubbing, weight loss, hyperhidrosis, pulmonary hypertension/restrictive lung disease, thrombotic diatheses, diarrhea, low vitamin B12 values |
| . | Criteria/other symptoms and signs . | Affected, %* . |
|---|---|---|
| Mandatory major criteria (both required) | 1. Polyradiculoneuropathy (typically demyelinating) | 100 |
| 2. Monoclonal plasma cell disorder (almost always λ) | 100† | |
| Other major criteria (1 required) | 3. Castleman disease‡ | 11-25 |
| 4. Sclerotic bone lesions | 27-97 | |
| 5. VEGF elevation§ | ||
| Minor criteria (1 required) | 6. Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy) | 45-85 |
| 7. Extravascular volume overload (edema, pleural effusion, or ascites) | 29-87 | |
| 8. Endocrinopathy (adrenal, thyroid,‖ pituitary, gonadal, parathyroid, pancreatic‖) | 67-84 | |
| 9. Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, white nails) | 68-89 | |
| 10. Papilledema | 29-64 | |
| 11. Thrombocytosis/polycythemia¶ | 54-88 | |
| Other symptoms and signs | Clubbing, weight loss, hyperhidrosis, pulmonary hypertension/restrictive lung disease, thrombotic diatheses, diarrhea, low vitamin B12 values |
The diagnosis of POEMS syndrome is confirmed when both of the mandatory major criteria, 1 of the 3 other major criteria, and 1 of the 6 minor criteria are present.
Summary of frequencies of POEMS syndrome features based on largest retrospective series.2,7-11
Takasuki and Nakanishi series are included, even though only 75% of patients had a documented plasma cell disorder. Because these are among the earliest series describing the syndrome, they are included.
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.
A plasma VEGF level of 200 pg/mL is 95% specific and 68% sensitive for a POEMS syndrome.28
Because of the high prevalence of diabetes mellitus and thyroid abnormalities, this diagnosis alone is not sufficient to meet this minor criterion.
Approximately 50% of patients will have bone marrow changes that distinguish it from a typical monoclonal gammopathy of undetermined significance or myeloma bone marrow.37