Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]
The diagnosis of POEMS syndrome presents a diagnostic challenge for the physician. A thorough examination of organ systems should be attempted in order to reach a confirmed diagnosis. POEMS syndrome should be differentiated from other conditions presenting as a polyneuropathy (metabolic syndrome, vitamin B12 deficiency, Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy and multifocal motor polyneuropathy), organomegaly with lymphadenopathy (malaria, leshmaniasis or kala-azar, infective hepatitis, chronic myelogenous leukemia, lymphoma, primary amyloidosis, Gaucher's disease), endocrinopathy (hypogonadism, hypothyroidism, hypopituitarism), monoclonal plasma cell proliferation (multiple myeloma, monoclonal gammopathy of undetermined significance, plasmacytoma), mixed lytic/sclerotic bone lesions (osteomalacia, osteogenesis imperfecta) and skin changes.
The diagnosis of POEMS syndrome presents a diagnostic challenge for the physician. A thorough examination of organ systems should be attempted in order to reach a confirmed diagnosis. POEMS syndrome should be differentiated from other conditions presenting as a polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell proliferation, mixed lytic/sclerotic bone lesions and skin changes. The differentials include the following:
Organ System Involvement | Differential Diagnosis | Causes | Clinical Features | Laboratory Findings | Gold Standard Test | Therapy | |
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P = Polyneuropathy | POEMS syndrome (Demyelinating)[1] |
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Metabolic Syndrome (Axonal pathology)[8] |
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Vitamin Deficiencies (Axonal Pathology)[10] |
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Guillain-Barre Syndrome (Demyelinating)[16] |
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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Mixed axonal and demyelinatiing)[24][25] |
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Multifocal Motor Neuropathy[35] |
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Organ System Involvement | Differential Diagnosis | Causes | Features | Laboratory Findings | Gold Standard Test | Therapy | |
O = Organomegaly (Hepatosplenomegaly and Lymphadenopathy) | Malaria[37][38][39] |
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Kala-azar[40][41] |
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Infective Hepatitis[42][43] |
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Chronic Myelogenous Leukemia (CML)[44][45][46][47][48][49][50][51][52][51] |
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Lymphoma |
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Primary (AL) Amyloidosis[54][55] |
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Gaucher's Disease[61] |
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Organ System Involvement | Differential Diagnosis | Causes | Features | Laboratory Findings | Gold Standard Test | Therapy | |
E = Endocrinopathy (Hypogonadism, Hypothyroidism, Hypopituitarism) | Hypogonadism[62][63] | Primary Hypogonadism (Hypergonadotrophic) |
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Secondary Hypogonadism (Hypogonadotrophic) |
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Hypothyroidism[64][65] | Primary Hypothyroidism |
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Secondary Hypothyroidism |
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Tertiary Hypothyroidism |
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Hypopituitarism[66][67] | Congenital |
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Acquired |
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Organ System Involvement | Differential Diagnosis | Causes | Features | Laboratory Findings | Gold Standard Test | Therapy | |
M = M-protein ( Hematological Abnormality/Plasma Cell Dyscrasias) | Multiple myeloma[68][69][70] |
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AND
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Monoclonal gammopathy of undetermined significance (MGUS)[71] |
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AND
AND
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Asymptomatic Plasma Cell Myeloma |
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OR
AND/OR
AND
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Plasmacytoma |
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Bone Lesions | Osteoporosis |
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Osteomalacia[72] |
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Osteogenesis imperfecta |
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Skin Changes | Scurvy |
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POEMS syndrome must also be differentiated from other similar conditions which lead to multiple endocrine disorders such as autoimmune polyendocrine syndrome, Hirata syndrome, Kearns–Sayre syndrome and Wolfram syndromes.[73][74][75][76][77]
Disease | Addison's disease | Type 1 diabetes mellitus | Hypothyroidism | Other disorders present |
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POEMS syndrome | + | Less common | Less common | Hypoparathyroidism Candidiasis Hypogonadism |
APS type 2 | + | + | + | Hypogonadism Malabsorption |
APS type 3 | - | + | + | Malabsorption |
Thymoma | + | - | + | Myasthenia gravis Cushing syndrome |
Chromosomal abnormalities (Turner syndrome, Down's syndrome) |
- | + | + | Cardiac dysfunction |
Kearns–Sayre syndrome | - | + | - | Myopathy Hypoparathyroidism Hypogonadism |
Wolfram syndrome | - | + | - | Diabetes insipidus Optic atrophy Deafness |
POEMS syndrome | - | + | - | Polyneuropathy Hypogonadism Plasma cell dyscrasias |