POEMS syndrome

POEMS syndrome is a paraneoplastic syndrome characterized by polyradiculoneuropathy (P), organomegaly (O), endocrinopathy (E), clonal plasma cell disorder (M), and skin changes (S). Other features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis, and elevated VEGF levels.

Hepatosplenomegaly

Simultaneous enlargement of the liver and spleen.


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PMID (PMCID)
28291506
 FEMALE Adult
Immunotactoid glomerulopathy leading to the discovery of POEMS syndrome
.
Philipponnet C, Kemeny JL, Garrouste C, Soubrier M, Heng AE.
Clin Nephrol. 2017;87(6):310-315.
At the same time, a POEMS syndrome diagnosis (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin abnormalities) was confirmed in light of the following: 1) IgG 954 monoclonal gammopathy, 2) axonal neuropathy, 3) osteosclerosis, 4) melanoderma, 5) hepatosplenomegaly and adenopathies, 6) Castleman disease, and 7) edema.
27401385
 FEMALE Adult
[Chronic kidney disease associated with Poems syndrome: Report of one case].
Vega J.
Rev Med Chil. 2016;144(4):516-20.
We report a woman with the syndrome, who had peripheral polyneuropathy, osteosclerotic myeloma, monoclonal IgA elevation, hypothyroidism, hypogonadotrophic hypogonadism, hyperprolactinemia, adrenal insufficiency, hepatosplenomegaly, lymphadenopathy, thyroid and parotid enlargement, Castlemans disease, papilledema, stiff and hyperpigmented skin, white nails, clubbing, ascites and chronic diarrhea.
25566064
(4280463)
 OTHER
Local Treatment with Triamcinolone Acetonide and Bevacizumab for Ocular Symptoms in a Patient with POEMS Syndrome.
Prost MG, Gilhuis HJ, Brouwer RE, Gawda P.
Case Rep Ophthalmol. 2014;5(3):416-22.
The diagnosis of POEMS syndrome was made based on the polyneuropathy, hepatosplenomegaly, IgA lambda monoclonal proteinemia, and optic disc swelling.
20513935
 FEMALE Middle Aged
POEMS syndrome: a rare case of monoclonal plasmaproliferative disorder.
Oehadian A, Prasetya D, Fadjari TH.
Acta Med Indones. 2010;42(2):100-3.
Although POEMS syndrome is a rare disease, it should be considered in patient with hepatosplenomegaly, especially if accompanied by peripheral neuropathy.
19670534
 MALE Child
Poems syndrome--a rare variant of plasma cell dyscrasia. Case report and review of literature.
Goranova-Marinova VS, Chernev KG, Goranov SE.
Folia Med (Plovdiv). 2009;51(2):5-11.
We present a case of POEMS syndrome in a 47-year-old male patient who initially presented with edema of the lower limbs, moderate lymphadenopathy, hepatosplenomegaly, histological findings of Castleman disease with marked sinusoidal angioproliferation in the lymph nodes and multiple osteosclerotic lesions.
18376288
 FEMALE
Case report: POEMS syndrome in childhood.
Sevketoglu E, Hatipoglu S, Ayan I, Dogan O, Salihoglu B.
J Pediatr Hematol Oncol. 2008;30(3):235-8.
We report a 15-year-old girl with POEMS syndrome, who developed growth retardation, delayed puberty, gradually increasing abdominal distention, brown skin pigmentation, hypogonadism, hepatosplenomegaly, lympadenomegaly, monoclonal gammopathy, and anemia.
16307346
 MALE Middle Aged
An atypical case of POEMS syndrome with IgG kappa M protein and end stage renal failure.
Dursun B, Artac M, Varan HI, Akkaya BK, Karpuzoglu G, Suleymanlar G.
Int Urol Nephrol. 2005;37(3):581-5.
Here, we presented an atypical case of POEMS syndrome with IgG kappa monoclonal protein, chronic demyelinating polyneuropathy, hepatosplenomegaly, hypothyroidism, gynecomastia and severe renal impairment.
15991840
 MIXED_SAMPLE Middle Aged
Idiopathic multicentric Castleman's disease. A clinicopathologic and immunohistochemical study of five cases.
Kojima M, Nakamura S, Nishikawa M, Itoh H, Miyawaki S, Masawa N.
Pathol Res Pract. 2005;201(4):325-32.
Some of the characteristic clinical findings of POEMS syndrome, including hepatosplenomegaly, skin change, endocrine abnormalities and anasarca, were present in all five cases.
15293761
 FEMALE Middle Aged
[A case of Crow-Fukase syndrome showing improvement following excision and irradiation of bone lesions].
Yoritaka A, Sakai M, Ohta K, Kishida S.
Rinsho Shinkeigaku. 2004;44(6):369-71.
A 57-year-old woman suffering from pleural and pericardial effusion, pulmonary hypertention, lymphadenopathy, hepatosplenomegaly, edema, hypertrichosis, small hemangioma and polyneuropathy was diagnosed as Crow-Fukase syndrome.
14615950
 MALE
[POEMS Syndrome with central and peripheric nervous system demyelination].
Masjuan Vallejo J, Herrero Valverde A, Mera Campillo J, Lopez Jimenez J.
Neurologia. 2003;18(8):465-9.
We report the case of a 67 years old patient who developed POEMS syndrome associated to Castleman's disease, characterized by demyelinating polyradiculoneuropathy, hepatosplenomegaly, mediastinal and supraclavicular adenopathies and monoclonal IgG lambda gammapathy.
1816211
 MALE Adult
POEMS syndrome.
Prasad K, Ahuja GK, Goulatia RK, Sood A, Dawar R, Behari M.
J Assoc Physicians India. 1991;39(10):780-2.
A patient with peripheral polyneuropathy, hepatosplenomegaly, osteosclerotic myeloma in the ilium, hyperpigmentation and hypertrichosis is described.
2276681
 MALE Middle Aged
POEMS syndrome: a case report.
Bisail M, Cossu A, Massarelli G, Tanda F, Agnetti V, Traccis S, Cassisa L, Puliga MV, Bartoli E.
Haematologica. 1990;75(4):384-6.
A case of POEMS syndrome in a 43-year-old male with polyneuropathy, osteolytic lesion of the basin due to solitary IgA-lambda plasmacytoma, cutaneous scleroderma-like changes, diffuse lymphadenopathy and hepatosplenomegaly is described.