Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A rare association of multisystemic manifestations with plasma cell dyscrasia has been termed POEMS syndrome, which includes polyneuropathy, organomegaly, endocrinopathy. M-protein, and skin changes. From literature reports, organomegaly consists of hepatosplenomegaly and/or lymphadenopathy. We here present a case of POEMS syndrome that had most of the typical features of POEMS, except that organomegaly only involved the heart. The patient's multiple clinical manifestations, including cardiomyopathy, improved with chemotherapy, which is characteristic of POEMS syndrome. However, cardiomegaly or cardiomyopathy have not previously been described with POEMS. The present case suggests that cardiomegaly and cardiomyopathy may be added to the organomegaly in POEMS.
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PMID:POEMS syndrome presenting with cardiomegaly and cardiomyopathy. 153 13

A patient with peripheral polyneuropathy, hepatosplenomegaly, osteosclerotic myeloma in the ilium, hyperpigmentation and hypertrichosis is described. A diagnosis of POEMS syndrome was made. This is the first report of this syndrome from India.
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PMID:POEMS syndrome. 181 11

POEMS (acronym for polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) is a very rare syndrome probably related to plasma cell dyscrasia. A 43 year old man developed a progressive symmetric sensory motor polyneuropathy 2 years before admission. Hepatosplenomegaly and sclerodermatoid skin changes were present on physical examination. A sclerotic lesion of the right femur was disclosed by radiologic examination. Serum immunoelectrophoresis demonstrated a monoclonal protein IgG-lambda pattern and the bone marrow biopsy revealed an increased plasma cell count (15%). The patient died 7 months after admission from pneumonia. A review of the literature is included.
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PMID:[Poems syndrome: review of a case]. 184 55

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. Liver biopsy showed a regenerative process of the parenchyma without laboratory and histologic evidence of necrosis. A peculiar finding was the onset of a right hemiparesis in the absence of signs of vascular disease or other predisposing factors. The possible links between the peculiar elements of the POEMS syndrome are briefly analyzed and discussed.
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PMID:POEMS syndrome: a case report. 227 81

POEMS (polyneuropathy, organomegaly [hepatosplenomegaly or lymphadenopathy], endocrinopathy, M protein, and skin changes) syndrome is an uncommon plasma cell dyscrasia with diverse manifestations, including lymphadenopathy as well as those that comprise the acronym. Dermatologic changes may include hyperpigmentation, thickened skin, hypertrichosis, and papular angiomas. These changes are believed to result from humoral products of the plasma cell clone and frequently resolve with treatment of the neoplasm. We describe a man with typical POEMS syndrome with a unique skin lesion: a large, sharply circumscribed, pigmented plaque on the anterior chest wall centered over a plasmacytoma of the sternum. Results of histologic examination showed a proliferation of capillaries of varying size, enlarged fibroblasts, and increased amounts of collagen and proteoglycan from the dermis to the periosteum. Because the outer cortex of the sternum was eroded, peptide mediators of vascular and fibroblast proliferation may have diffused directly from the plasma cell lesion into the tissues of the chest wall.
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PMID:Plasma cell dyscrasia: a case of POEMS syndrome with a unique dermatologic presentation. 268 Dec 92

A patient with osteosclerotic myeloma and POEMS syndrome, unresponsive to pulse prednisone and melphalan therapy, was admitted to the hospital for a trial of plasma exchange therapy. The presentation included IgG lambda monoclonal gammopathy, peripheral neuropathy, hepatosplenomegaly, hyperpigmentation and thickening of the skin, edema, and tense ascites. Laboratory tests confirmed hypothyroidism, hypogonadism, and adrenal insufficiency. Six exchange procedures failed to affect the clinical course, and the patient died. Greater-than-one-plasma-volume exchanges (patient's measured plasma volume, 2,703 cc) were performed. When IgG and cholesterol removal were compared to the predicted removal, based on the volume of plasma removed, significantly less reduction in concentration than predicted was measured. IgG concentrations increased postapheresis and, at 2 weeks, three-fourths of the removed IgG had reaccumulated. A reduced efficiency of removal of both IgG and cholesterol can be explained by postulating increased vascular permeability with free exchange of soluble substances from one compartment to another. If an abnormal product is produced by the disease and is responsible for the clinical syndrome, a more intensive schedule of plasma exchange therapy may be needed to achieve a sustained depletion of the responsible soluble substance. Alternatively, neither increased vascular permeability or the clinical manifestations are responsive to removal of a soluble substance or are caused by a soluble substance produced by the malignancy.
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PMID:Therapeutic trial of plasma exchange in osteosclerotic myeloma associated with the POEMS syndrome. 299 55

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

Three patients with an unusual multisystemic syndrome characterized by polyneuropathy, organomegaly *especially hepatosplenomegaly), endocrine dysfunction, M-protein, and skin abnormalities (POEMS syndrome) are discussed. Characteristic radiographic features include single or multiple osteosclerotic lesions and peculiar variety of bony proliferation. The relationship of the disorder to multiple myeloma and plasmacytoma and the pathogenesis of the skeletal abnormalities remain obscure.
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PMID:Plasma-cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes: the POEMS syndrome. Distinctive radiographic abnormalities. 724 23

Crow-Fukase syndrome is a rare multiorgan disorder. Although renal disorders, such as proteinuria, and renal impairment, have been observed in half the cases of this syndrome, there have been few reports describing the renal lesions. We report here a case of this syndrome associated with membranoproliferative glomerulonephritis. A 43-year-old woman was referred to our hospital because of hyperglycemia. She had also been suffering from hyperpigmentation, hepatosplenomegaly, lymphadenopathy, polyneuropathy and endocrine dysfunction, including diabetes mellitus and amenorrhea. Serum electrophoresis showed M protein and immunoelectrophoresis revealed IgA (lambda). Bone marrow aspiration showed a slight increase in the number of plasma cells. Urine protein was 30 mg/dl, BUN was 17 mg/dl and creatinine 0.8 mg/dl. Light microscopic examinations showed enlargement of glomeruli with proliferation of mesangial cells and matrix, a lobular pattern of the glomeruli and thickening of the glomerular basement membrane and associated double contour. Electron microscopic examinations showed thickened capillary walls, associated mesangial interposition and subendothelial dense deposits. Moreover, fine granular deposits of IgM, C3, and fibrinogen along the basement membrane were observed on immunofluorescent studies.
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PMID:[A case of Crow-Fukase syndrome associated with membranoproliferative glomerulonephritis]. 807 25


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