No abstract available.
POEMS Syndrome*

No abstract available.
POEMS Syndrome* ; Stroke*

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) also known as Crow-Fukase syndrome or Takatsuki syndrome is a rare and disabling paraneoplastic syndrome that frequently occurs in the fifth or sixth decade of life without a known standard first-line therapy. A 34-year-old Filipino male nurse, who presented with gradually progressive distal lower extremity weakness and sharp shooting pain in bilateral legs accompanied by mild joint effusion on the left knee, hypertrichosis, bilateral conjunctival injection, and gynecomastia underwent extensive workup and was diagnosed with POEMS syndrome. Complete blood count revealed erythrocytosis and thrombocytosis with elevated serum VEGF (vascular endothelial growth factor) and elevated monoclonal serum free lambda light chains. The electrophysiologic studies revealed chronic demyelinating sensorimotor polyneuropathy while bone marrow core biopsy and bone marrow aspirate smear immunohistochemical staining showed it to be positive for lambda and CD138. He had an initial unsuccessful treatment course with melphalan and prednisone. Hence, bortezomib and dexamethasone were given which gave significant improvement in symptoms from the overall neuropathy limitation score of 5 to 1.

Humans ; POEMS Syndrome

No abstract available.
Early Diagnosis* ; Hemangioma* ; POEMS Syndrome*

No abstract available.
Humans ; POEMS Syndrome* ; Pulmonary Edema*

Humans ; POEMS Syndrome ; diagnosis ; therapy

Two patients with an unusual multisystemic syndrome characterized by polyneuropathy, organomegaly(especiallyhepatosplenomegaly), endocrine dysfunction, M-protein, and skin abnormalites(POEMS syndrome) are discussed.Characteristic radiographic features include hepatosplenomegaly, lymph node enlargement, sclerotic bony lesions,and a peculiar variety of bony proliferation.
Humans ; Lymph Nodes ; POEMS Syndrome* ; Polyneuropathies ; Skin

OBJECTIVETo evaluate the diagnostic performance and clinical correlation of serum vascular endothelial growth factor (VEGF) in patients with newly diagnosed POEMS syndrome.

METHODSWe recruited 104 patients with POEMS syndrome who diagnosed at Peking Union Medical College Hospital during October 2010 to April 2014 in the current study. Clinical data and serum samples were collected. Serum VEGF levels were measured, comparing to both disease and healthy controls, and its correlation with clinical features were analyzed.

RESULTSPatients with newly diagnosed POEMS syndrome showed markedly elevated levels of serum VEGF (median 3503 ng/L, P<0.01). A cut-off value of 1 200 ng/L had a specificity of 90.2%, with a sensitivity of 83.7%, in support of a POEMS diagnosis. Further analyses indicated that patients with higher serum VEGF level (>2 000 ng/L) had more prevalent osteosclerosis (61.0% vs 37.0%, P=0.031) and papilledema (70.1% vs 44.4%, P=0.017).

CONCLUSIONSerum VEGF level was useful in differentiating POEMS syndrome from other disorders with overlapping clinical presentations, and its levels correlated with several disease features.

Renal involvements of POEMS syndrome are not rare. In some reports, almost 50% of patients show proteinuria. There are some case reports of renal involvement in POEMS syndrome in Korea, but there are no reports about clinical features of renal involvement in POEMS syndrome in Korea and its frequency. We report a case of POEMS syndrome with nephropathy and clinical features of renal involvement in POEMS syndrome in Korea. The most frequent symptoms in POEMS syndrome in Korea were polyneuropathy, edema and ascites. Renal involvement was found in 40% of patients. If there were unknown cause of edema and renal failure when combined with polyneuropathy, considerations should be taken into patients for POEMS syndrome even though POEMS syndrome is very rare.
Ascites ; Edema ; Humans ; Korea* ; POEMS Syndrome* ; Polyneuropathies ; Proteinuria ; Renal Insufficiency