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.

Splenomegaly

Abnormal increased size of the spleen.


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PMID (PMCID)
29893339
 MALE Middle Aged
An atypical presentation of POEMS syndrome with IgG kappa type M protein and normal VEGF level: Case report and review of literature.
Pulivarthi S, Gurram MK.
J Cancer Res Ther. 2018;14(3):679-681.
We present an atypical case of POEMS syndrome with subclinical polyneuropathy, splenomegaly, hypogonadism, and myeloma of immunoglobulin G kappa type.
29113155
(5656028)
 OTHER
POEMS syndrome presentation with progressive weakness in upper and lower limbs: A case report.
Cao CJ, Dou CY, Zhou KH, Liu JB, Lai H.
Oncol Lett. 2017;14(5):5197-5202.
A 50-year-old female patient presented with progressive weakness in her upper and lower limbs; tingling, numbness and burning in her feet; polyneuropathy (demyelinating in the majority of cases of POEMS syndrome); monoclonal plasma cell disorder (typically955-restricted in cases of POEMS syndrome); sclerotic lesions on the spine and pelvis; organomegaly, including hepatomegaly, splenomegaly and lymphadenopathy; edema; pleural effusion; adrenal, thyroidal, pituitary, gonadal and pancreatic endocrinopathy; skin changes, including hyperpigmentation, dry skin and hypertrichosis; thrombocytosis; pulmonary hypertension; low vitamin B12 and weight loss.
29113155
(5656028)
 OTHER
POEMS syndrome presentation with progressive weakness in upper and lower limbs: A case report.
Cao CJ, Dou CY, Zhou KH, Liu JB, Lai H.
Oncol Lett. 2017;14(5):5197-5202.
A 50-year-old female patient presented with progressive weakness in her upper and lower limbs; tingling, numbness and burning in her feet; polyneuropathy (demyelinating in the majority of cases of POEMS syndrome); monoclonal plasma cell disorder (typically955-restricted in cases of POEMS syndrome); sclerotic lesions on the spine and pelvis; organomegaly, including hepatomegaly, splenomegaly and lymphadenopathy; edema; pleural effusion; adrenal, thyroidal, pituitary, gonadal and pancreatic endocrinopathy; skin changes, including hyperpigmentation, dry skin and hypertrichosis; thrombocytosis; pulmonary hypertension; low vitamin B12 and weight loss.
20403034
 FEMALE Adult
POEMS syndrome with idiopathic portal hypertension: autopsy case and review of the literature.
Inoue R, Nakazawa A, Tsukada N, Katoh Y, Nagao T, Nakanuma Y, Mukai K.
Pathol Int. 2010;60(4):316-20.
Diagnosis of POEMS syndrome was established on additional symptoms (splenomegaly and papilloedema) and serum M-protein.
17040261
 MALE Adult
Glomeruloid haemangioma with cerebriform morphology in a patient with POEMS syndrome.
Chan PT, Lee KC, Chong LY, Lo KK, Cheung YF.
Clin Exp Dermatol. 2006;31(6):775-7.
After chemotherapy, other manifestations of POEMS syndrome developed, such as multiple haemangiomas over the chest and neck region, splenomegaly and generalized oedema.
16926143
 MALE Adult
Successful treatment of a patient with POEMS syndrome by tandem high-dose chemotherapy with autologous CD34+ purged stem cell rescue.
Kojima H, Katsuoka Y, Katsura Y, Suzuki S, Suzukawa K, Hasegawa Y, Nagasawa T.
Int J Hematol. 2006;84(2):182-5.
Based on the existence of monoclonal gammopathy, the proliferation of abnormal plasma cells in the bone marrow, the presence of sclerotic bone lesion, polycythemia, mild splenomegaly, and an elevated level of serum vascular endothelial growth factor (VEGF) (14,900 pg/mL; normal, 62-707), he was diagnosed as having peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome.
15884463
 MALE Middle Aged
Polyneuropathy with osteosclerotic myeloma--POEMS syndrome.
Kumar P, Verma A, Kishore D, Parhi LD, Joshi D, Misra S.
J Assoc Physicians India. 2004;52:997-8.
A 55-years-old male, who presented with insidious onset gradually progressive sensorimotor polyneuropathy, POEMS-syndrome was diagnosed based on polyneuropathy, splenomegaly, hypothyroidism, the presence of IgG-monoclonal serum protein with osteosclerotic lesions and hyperpigmention of skin.
11876037
 MALE Middle Aged
[A man with plasma cell dyscrasia and polyneuropathy due to POEMS syndrome].
Driessens SK, Wildiers H, Verhoef GE, Vanstraelen D, Robberecht W, Vandenberghe P.
Ned Tijdschr Geneeskd. 2002;146(7):321-5.
In a 52-year-old man with general malaise, muscle stiffness and weakness, POEMS-syndrome was diagnosed based on polyneuropathy, splenomegaly, lymphadenopathy, subclinical hypothyroidism and the presence of a monoclonal paraprotein with osteosclerotic lesions and an indurated skin (POEMS is an acronym for Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes).
2227242
 FEMALE Adult
[Unusual cause of ascites: the POEMS syndrome].
Berson A, Fabre M, Buffet C, Rougier P, Guillaume JC, Venencie PY, Schaison G, Pateron D, Charpentier P, Etienne JP.
Gastroenterol Clin Biol. 1990;14(8-9):675-9.
A 39-year-old woman presented with polyneuropathy, hepatomegaly, splenomegaly, endocrinopathy, monoclonal protein and skin changes, several of the many clinical features of the recently described POEMS syndrome.
2537136
 MALE Adult
POEMS syndrome associated with polycythemia vera.
Schulz W, Domenico D, Nand S.
Cancer. 1989;63(6):1175-8.
The authors describe a patient with POEMS syndrome who has polyneuropathy of the upper and lower extremities, splenomegaly, impotence, IgA-lambda monoclonal protein (M-protein), and marked thickening of his skin.