Cases reported "POEMS Syndrome"

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1/131. poems syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia.

    poems syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, poems syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically. Skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced. However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that poems syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia. ( info)

2/131. poems syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma.

    poems syndrome is a rare condition with cutaneous manifestations commonly including angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphadenopathy, erythematous thickening of the skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring alopecia and sparse follicular pustules at the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monoclonal gammopathy, subclinical thyreopathy, and an osteolytic bone lesion of the skull. Histologically, a plasmacytoma with lambda cell restriction was found. The overlying skin showed marked fibrosis, with loss of hair follicles, and a plasma cell infiltrate of polyclonal origin. The cervical lymph nodes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There was no evidence of further plamacytomas. After excision of the plasmacytoma and postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further folliculitis or hair loss on the rest of the scalp. This case illustrates the reactive character of poems syndrome as a paraneoplastic syndrome in myeloma patients. ( info)

3/131. Pulmonary manifestations of poems syndrome: case report and literature review.

    phrenic nerve paresis is an unusual complication of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein spike and skin changes) syndrome. In this report, we describe a case of poems syndrome in which a 56-year-old woman presented with dyspnea and ventilatory failure due to bilateral phrenic nerve paralysis. To our knowledge, only one other case of phrenic neuropathy in poems syndrome has been reported. ( info)

4/131. Accelerated conversion of dehydroepiandrosterone sulfate to estrogen in a patient with Crow-Fukase syndrome and diabetes mellitus.

    About 28% of patients with the Crow-Fukase syndrome exhibit glucose intolerance which may be induced by low serum levels of dehydroepiandrosterone (DHEA). We report a patient with the Crow-Fukase syndrome who exhibited non-insulin dependent diabetes mellitus (NIDDM) worsened prior to admission. He received the DHEA sulfate (DHEA-S) infusion test to evaluate aromatase activity. This patient exhibited an increase in aromatase activity measured by the conversion of the intravenously loaded DHEA-S to estrogen, and low serum levels of DHEA and DHEA-S. These abnormalities returned to nearly normal during the administration of prednisolone, 60 mg per day. No adverse effect on his diabetes was observed during the corticosteroid treatment. Five control patients with diabetes but without the Crow-Fukase syndrome showed no increase in the conversion of DHEA-S to estrogen, which suggests that aromatase activity is normal in diabetes. The increase in aromatase activity in our patient may have led to a low serum concentration of DHEA that in turn caused glucose intolerance and a deterioration of the diabetes prior to admission. Glucocorticoid therapy may be beneficial in Crow-Fukase syndrome to improve the distorted metabolism of DHEA with no adverse effect on the diabetes. ( info)

5/131. poems syndrome with xanthomatous cells. Polyneuropathy Organomegaly Endocrinopathy M-protein Skin changes.

    A diffuse xanthomatous infiltration was detected on a biopsy of the hyperpigmented patches in a 40-year-old man with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes) syndrome. Multiple biopsies of the hyperpigmented patches showed diffuse or perivascular foamy histiocytes in the upper and mid dermis, a sparse infiltrate of lymphocytes, fibrosis, hyperpigmentation of the basal layer, and capillary proliferation. The foamy cells were positive for periodic acid-Schiff (PAS) with diastase, oil red-O, and CD68. To our knowledge, this is the first case with foamy histiocytes without xanthomatous-appearing skin lesions in poems syndrome. It would be worth searching for xanthomatous histiocytes in the hyperpigmented skin of patients with poems syndrome. ( info)

6/131. Soluble immune mediators in poems syndrome with pulmonary hypertension: case report and review of the literature.

    poems syndrome is a plasma cell dyscrasia that presents with numerous complications, one of which is rarely pulmonary hypertension. Here we present a case of poems syndrome with pulmonary hypertension who improved with steroids and six rounds of plasmapheresis done over 1 month, and we document the baseline immune mediator status and the changes associated with the therapeutic intervention. serum levels of soluble immune mediators such as interleukin (IL)-5, IL-8, IL-10, and eotaxin were normal at baseline and throughout therapy, whereas those of tumor necrosis factor (TNF)-alpha, soluble TNF-receptor type I (sTNF-RI), IL-6, interferon (IFN)-gamma, IL-2, and sIL-2R, which were abnormally high at baseline normalized with steroids and plasmapheresis. serum levels of sIL-6R, which were abnormally low at baseline, increased to normal after therapy. The latter results pinpoint not only potential mediators of the systemic manifestations of poems syndrome with pulmonary hypertension but also relevant markers in patient follow-up. In this respect, IL-6 has been involved in the pathogenesis of multiple myeloma and Castleman's disease, and the interplay between abnormally high levels of IL-6 and abnormally low levels of its soluble receptor, deficiencies that corrected with therapy in this patient, appears to be particularly relevant to the pathogenic manifestations of poems syndrome with pulmonary hypertension. These findings are discussed in the context of our current knowledge of the pathogenesis of pulmonary hypertension and of potential new therapeutic modalities for poems syndrome with pulmonary hypertension. ( info)

7/131. Glomeruloid hemangioma in poems syndrome shows two different immunophenotypic endothelial cells.

    The case of a Japanese woman with glomeruloid hemangioma, an initial marker for poems syndrome, is reported. Her cutaneous lesions were multiple and consisted of glomeruloid hemangiomas, cherry-type capillary hemangiomas, and a mixture of both. The specimens of glomeruloid hemangiomas were studied by paraffin section immunohistochemistry with a large panel of antibodies and electron microscopy, respectively. The lesions, whose size ranged from minute foci to large nodules, were composed of anastomosing vascular channels resembling renal glomeruli and had irregular lumina, often featuring capillaries and sinusoid-like spaces. The vascular channels were lined by a single layer of endothelial cells, which showed two types of cells. The capillary-type endothelium possessed large vesicular nuclei with open chromatin and large amount of cytoplasm. The sinusoidal endothelium possessed small basal nuclei with dense chromatin as well as scant amount of cytoplasm. The former cells had a characteristic CD31 /CD34 /UEA I /CD68- phenotype. Some of these cells ultrastructurally showed intracytoplasmic lumen formation. The latter cells had a characteristic CD31 /CD34-/UEA I-/CD68 phenotype. The present study shows that glomeruloid hemangioma has unique morphologic and immunologic features that differ from the traditional hemangiomas as well as littoral cell angioma of the spleen. ( info)

8/131. Kaposi's sarcoma herpesvirus-associated Castleman's disease with poems syndrome.

    The pathogenic link between POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes, and various other clinical signs) syndrome and Castleman's disease is unclear. Roles for M protein in poems syndrome and cytokines in systemic manifestations of multicentric Castleman's disease have been suggested. Recently, pathogenic roles of cytokines in poems syndrome and Kaposi's sarcoma-associated herpesvirus (KSHV) in Castleman's disease have been reported. We report on a patient with KSHV-associated Castleman's disease with poems syndrome, and suggest a possible role of KSHV in initiating and linking these two diseases. ( info)

9/131. A man with worsening weakness.

    The contemporary presence of organomegaly, skin manifestations, polyneuropathy, endocrinopathy and monoclonal component characterises the poems syndrome, often associated with osteosclerotic myeloma and Castelman's disease and more frequent in the Japanese. Clinical manifestations seem to be related to the production of many interleukins, mainly IL-1, IL-6 and TNF. Several endocrinopathies have been described, the most frequent being diabetes. Only one previous case of hypoparathyroidism associated with the syndrome has been described in medical reviews. Polyneuropathy is often sensitivo-motory and skin disease accounts for Raynaud phenomenon, skin pigmentation, hypertricosis and others. We describe the case of a 74-year-old man who underwent clinical examination for weakness mainly in the legs. Clinical and instrumental data showed rhabdomyolysis due to hypoparathyroidism. The contemporary presence of a monoclonal band of light chains on proteic electrophoresis, organomegaly and distal leg neuropathy allowed us to make a diagnosis of poems syndrome. ( info)

10/131. Reversible pulmonary hypertension in poems syndrome--another etiology of triggered pulmonary vasculopathy?

    Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome, a plasma cell dyscrasia associated with pulmonary hypertension, has been treated in the past with anticytokine strategies with a poor outcome. A patient is described who had poems syndrome with precapillary pulmonary hypertension and Raynaud's phenomenon, and who responded well to a short course of corticosteroids and long term nifedipine. poems syndrome, like anorexigens, cocaine, portal hypertension and human immunodeficiency virus infection, may be another trigger for the pulmonary vascular occlusive process found in primary pulmonary hypertension. ( info)
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