Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0152025 (polyneuropathy)
 7,862 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three patients with POEMS syndrome were studied: two of them had myeloma, whereas the third had M protein without myeloma. Peripheral nerve biopsies showed no deposits of anti-Ig sera at direct immunopathological examination. Ultrastructural study revealed myelino-axonal degeneration in all three cases. This was associated in the first case with a dramatic loss of myelinated fibers and there were lesions consistent with acute degeneration in the second case. In addition, all three had some fibers showing uncompacted myelin lamellae. This peculiar modification has previously been reported only in cases of dysglobulinemia, lymphoma and inflammatory demyelinating polyneuropathy, but its mode of formation remains unclear.
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PMID:Uncompacted lamellae in three patients with POEMS syndrome. 304 Apr 18

A patient with the syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) had multiple myeloma and IgA-kappa monoclonal gammopathy. To our knowledge, of over 50 cases in the literature, this is the only instance of kappa light chains noted in a patient with the complete syndrome. Sural nerve biopsy revealed increased immunofluorescence for IgA and kappa chains. Furthermore, this patient had a hemorrhagic diathesis characterized by a prolonged bleeding time and a prolonged thrombin time due to a qualitative platelet dysfunction and an apparent inhibition of fibrin monomer polymerization. In none of the other cases of POEMS syndrome was a coagulopathy reported. A short course of plasmapheresis improved the bleeding time but did not alter the polyneuropathy.
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PMID:Multiple myeloma with polyneuropathy and coagulopathy. A case report of the polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin change (POEMS) syndrome. 308 99

The case is reported of a 32-year-old woman with polyneuropathy, organomegaly, edema, endocrinopathy, dark skin and solitary myeloma with monoclonal gammopathy of IgG/lambda type. More than 100 cases of this rare disorder have been observed up to now, mainly in Japan. In recent reviews the terms POEMS-syndrome or Crow-Fukase-syndrome have been used. Local radiotherapy of the myeloma led to lasting regression of symptoms. Studies with immunocytochemistry and immunoelectroblotting revealed specific antibody activity against hypophysis, suggesting that the pathologic monoclonal myeloma antibodies may damage the neuroendocrinic centers in hypothalamus and hypophysis as the primary target. Most of the multisystemic symptoms would thus be explained as secondary alterations.
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PMID:[Polyneuropathy, organomegaly, endocrinopathy and skin changes in a case of solitary myeloma]. 309 18

POEMS syndrome is an unusual plasma cell dyscrasia with multisystemic manifestations featuring polyneuropathy, organomegaly, endocrinopathy, M protein and constant skin involvement. We have seen a 58-year-old white man presenting with generalized eruptive angiomas as a rare skin manifestation of this syndrome. We review the literature and discuss the pathogenesis of vascular proliferation in this unusual entity.
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PMID:Cutaneous angiomas in POEMS syndrome. 315 80

Cutaneous manifestations of POEMS (polyneuropathy, organomegaly, endocrinopathies, M-protein, and skin changes) syndrome in a 51-year-old white man included two types of hemangiomas, ie, cherry-type and subcutaneous hemangiomas. By light microscopy and immunohistochemistry, both types of tumors showed features of capillary hemangiomas. By electron microscopy, a peculiar finding of subcutaneous hemangiomas was the presence of slender abluminal processes of endothelial cells that were frequently anastomosing to form electron-lucent blebs. Although the precise etiopathogenesis of the vascular proliferations remains speculative, these lesions merit study since they constitute an easily recognizable marker of POEMS syndrome.
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PMID:Cutaneous angiomas in POEMS syndrome. An ultrastructural and immunohistochemical study. 328 67

A rare form of plasma cell dyscrasia characterized by the various association of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes has been termed POEMS syndrome. The pathogenesis of the multisystemic features of this syndrome remains unclear. Herein is reported a case of POEMS syndrome with striking clinical similarities with scleroderma, and microangiopathic glomerular lesions, as well as diffuse perivascular non-amyloid deposits, which could explain certain features of the syndrome, including peripheral nerve demyelination. It is proposed that a pathogenic role might be played by a non-immunoglobulin vasculotoxic component.
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PMID:POEMS syndrome presenting as systemic sclerosis. Clinical and pathologic study of a case with microangiopathic glomerular lesions. 334 53

A 58-year-old woman presented with a history of premature onset of menopause, longstanding hepatosplenomegaly, monoclonal gammopathy, lower limb polyneuropathy of recent onset, diabetes mellitus, excessive perspiration and leg edema. Polyneuropathy and excessive perspiration improved following a course of prednisone and melphalan. The clinical and pathophysiological features fit the rare entity known as POEMS syndrome.
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PMID:An unusual case of POEMS syndrome. 357 Jul 37

A 38-year-old black man with giant lymph node hyperplasia (GLH), osteoblastic lesions, and the POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) was treated at the University of Chicago Hospitals. The patient had hepatosplenomegaly and generalized peripheral lymphadenopathy. Endocrinologic abnormalities included decreased testosterone with elevated luteinizing hormone and follicle-stimulating hormone, as well as hyperprolactinemia and possible hypothyroidism. Biopsy of a right femoral lymph node revealed GLH, and an osteoblastic pelvic lesion showed a marked lymphoplasmacytic infiltrate. By immunohistochemical techniques, plasma cells in the lymph node and osteoblastic lesion were polyclonal. A polyclonal hypergammaglobulinemia was present. The lymph node T-lymphocyte population showed a decreased helper-to-suppressor cell ratio. Other findings included thickening of the skin, finger clubbing, and anasarca. A severe sensory-motor polyneuropathy was the major factor contributing to the patient's death. The association of GLH, osteoblastic bone lesions, and the POEMS syndrome has been noted previously in Japan; however, the authors are unaware of reports on Western patients who had this combination of clinical and laboratory findings.
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PMID:Giant lymph node hyperplasia with osteoblastic bone lesions and the POEMS (Takatsuki's) syndrome. 389 Oct 64

We studied a patient with POEMS syndrome (plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal [M]-protein, skin changes) who was also found to have renal enlargement and microangiopathic glomerulopathy. The latter finding is unusual in POEMS syndrome patients, and the associated symptoms can lead to confusion in diagnosis. We emphasize the importance of considering POEMS syndrome in the differential diagnosis of patients who have unexplained neuropathy, scleroderma-like skin changes, and/or monoclonal gammopathy.
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PMID:POEMS syndrome. Study of a patient with proteinuria, microangiopathic glomerulopathy, and renal enlargement. 395 5

Three cases of a unique multisystemic syndrome with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (the POEMS syndrome) are presented, along with a review of the literature. Clinical and radiographic features of this syndrome and etiological considerations are discussed. A variety of osteosclerotic lesions, nonspecific pleural effusion and ascites are characteristic radiographic manifestations.
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PMID:The POEMS syndrome: report of three cases with radiographic abnormalities. 650 18


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