Clinical findings of POEMS syndrome

Characteristic	% Affected*
Polyneuropathy	100
Organomegaly	45 to 85
Hepatomegaly	23 to 78
Splenomegaly	22 to 70
Lymphadenopathy	26 to 74
Castleman disease	11 to 25
Endocrinopathy	67 to 89
Gonadal axis abnormality	55 to 89
Adrenal axis abnormality	16 to 33
Increased prolactin value	5 to 20
Gynecomastia or galactorrhea	12 to 18
Diabetes mellitus	3 to 36
Hypothyroidism	9 to 67
Monoclonal plasma cell dyscrasia^¶	100
M-protein on serum protein electrophoresis	24 to 55
Skin changes	68 to 93
Hyperpigmentation	25 to 93
Acrocyanosis and plethora	19 to 46
Hemangioma/telangiectasia	9 to 35
Hypertrichosis	20 to 74
Thickening	5 to 43
Papilledema	29 to 64
Extravascular volume overload	29 to 89
Peripheral edema	24 to 89
Ascites	6 to 54
Pleural effusion	3 to 43
Pericardial effusion	1 to 64
Bone lesions	27 to 97
Thrombocytosis	34 to 88
Polycythemia	12 to 19
Clubbing	5 to 49
Decreased DLCO	>15
Pulmonary hypertension	36
Weight loss >10 lb	37 to 41
Fatigue	22 to 31

POEMS: polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes; DLCO: diffusing capacity of carbon monoxide.

* Percentages are based on the total number of patients in the series.

¶ In both the Takasuki and Nakanishi series, only 75% of patients had a documented plasma cell disorder, which defies the current definition for POEMS syndrome. As these are among the earliest series describing the syndrome, they are included.

References:

Takatsuki K, Sanada I. Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases. Jpn J Clin Oncol 1983; 13:543.
Nakanishi T, Sobue I, Toyokura Y, et al. The crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 1984; 34:712.
Soubrier MJ, Dubost JJ, Sauvezie BJ. POEMS syndrome: a study of 25 cases and a review of the literature. French study group on POEMS syndrome. Am J Med 1994; 97:543.
Li J, Zhou DB, Huang Z, et al. Clinical characteristics and long-term outcome of patients with POEMS syndrome in China. Ann Hematol 2011; 90:819.
Dispenzieri A, Kyle RA, Lacy MQ, et al. POEMS syndrome: definitions and long-term outcome. Blood 2003; 101:2496.
Ghandi GY, Basu R, Dispenzieri A, et al. Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. Mayo Clin Proc 2007; 82:836.
Keddie S, Foldes D, Caimari F, et al. Clinical characteristics, risk factors, and outcomes of POEMS syndrome: A longitudinal cohort study. Neurology 2020; 95:e268.

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Clinical findings of POEMS syndrome