Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A rare form of plasma cell dyscrasia characterized by associated polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes has been termed the POEMS syndrome. The pathophysiology is unknown; plasma cell dyscrasia is essential; secondary manifestations are unexplained. We report a 67-year-old man with a 7-month history of progressive weakness and numbness of the legs. Clinical examination revealed sensorimotor polyneuropathy, predominantly affecting the lower extremities, hepatomegaly, and skin haemangiomas. Additional investigations disclosed IgG-lambda monoclonal serum protein, endocrine abnormalities, elevated cerebrospinal fluid protein level and an osteoblastic lesion of the lumbar vertebra. Biopsy of the osteosclerotic vertebra showed a marked lymphoplasmocytic infiltrate. MRI of the liver disclosed two haemangiomas; this association has not been reported previously.
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PMID:Polyneuropathy with osteosclerotic myeloma--POEMS syndrome. A case report. 154 70

A 40 year old Chinese woman presented with anasarca and later developed features of POEMS syndrome. These are peripheral neuropathy (P), organomegaly (O), endocrine dysfunction (E), monoclonal gammopathy (M), and skin changes (S) which are usually associated with plasma cell dyscrasia. In our patient, monoclonal gammopathy was not detected on immunofixation electrophoresis and was revealed only after analysis of kappa/lambda light chain ratio of the raised serum IgA immunoglobulin. Needle liver biopsy of her grossly enlarged liver showed marked accumulation of glycogen and presence of giant mitochondria in the hepatocytes, a feature not previously reported.
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PMID:Poems syndrome. 174 70

A 47 years old male with POEMS syndrome is presented. His illness started seven months before admission. He was in poor general conditions and had malnutrition. He had cutaneous hyperpigmentation, skin changes, hepatomegaly, edema of the limbs, quadriplegia, myotatic reflexes abolished, hypotrophy of muscular masses and bilateral papilledema. Immunoglobulins IgA and IgM were abnormal. Bone scanning showed multiple lytic lesions. The study of bone marrow showed megaloblastic changes. Electromyography revealed severe neuropathy and biopsy from sural nerve showed severe demyelinization. At first, he showed improvement with steroids; nevertheless he died two months afterwards from possible bronchoaspiration. The autopsy revealed changes compatible with the clinical diagnosis and in addition an adenoma of the hypophysis. We emphasize the importance of POEMS syndrome in the differential diagnosis of polyneuropathies.
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PMID:[POEMS syndrome. Report of a case and review of the literature]. 148 88

This report deals with a case of double gammopathy (IgM-kappa, IgG-lambda) with Crow-Fukase syndrome, which developed into primary macroglobulinemia four years after the diagnosis. In May 1980, a 74-year-old woman was admitted to the hospital because of a rapid progression of peripheral neuropathy. The patient was diagnosed as having Crow-Fukase syndrome from the following data: albumin-cytologic dissociation of cerebrospinal fluid, peripheral edema, diffuse hyperpigmentation of the skin, diabetic glucose intolerance, serum double gammopathy (IgM-kappa, IgG-lambda) and hepatomegaly. The administration of prednisolone yielded the improvement of neuropathy. In December 1984, serum IgM level was increased from 104 mg/dl to 3,025 mg/dl. Plasma cells in the bone marrow increased in the percentage from 5.6% to 18.4%, and then Bence Jones protein (kappa type) was excreted in the urine. No antibody activity to myelin antigens was detected in the serum. The patient died of cerebral infarction in 1985. At postmortem examination, lymphomatous involvement was found in the jejunum. At the immunohistological examination of the tumor specimens, the morphology and the distribution of IgM- and IgG-positive cells corresponded to that of kappa- and lambda-positive cells, respectively. A small number of cells containing both kappa and lambda light chains were also demonstrated. It seems likely that IgM (kappa)- and IgG (lambda)-positive cells were derived from the common precursor cells.
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PMID:[Progression from Crow-Fukase syndrome with double gammopathy (IgM-kappa, IgG-lambda) to primary macroglobulinemia]. 194 31

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. In addition, she had a Castleman's disease (angiofollicular lymph node hyperplasia). In this case ascites was a main presenting feature. Thus, the POEMS syndrome must be added to the list of rare causes of ascites. Electron microscopy of the liver showed perisinusoidal fibrosis.
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PMID:[Unusual cause of ascites: the POEMS syndrome]. 222 42

A Thai male, aged 36 yrs, presented with chronic progressive sensorimotor polyneuropathy and mixed osteosclerotic and lytic lesions at the lumbar vertebrae (L). Generalized hyperpigmentation, bilateral gynecomastia, small testes, hepatomegaly, lymphadenopathy and ascites were observed. Computerized tomography of the lumbosacral spines revealed osteosclerotic changes at L3,4,5 with spiculated bony proliferation at the transverse processes and posterior components of the spines. Histopathological examination of the lesion was compatible with plasmacytoma. Proliferative vasculopathy was demonstrated in plasmacytoma. Serum immunofixation showed an IgM monoclonal gammopathy. Further investigation demonstrated secondary adrenal insufficiency with low level of plasma testosterone. The patient responded well to local radiotherapy and intermittent chemotherapy with melphalan and prednisolone. The histopathological findings of proliferative vasculopathy suggested the important role of capillary leakage as a factor responsible for systemic manifestations in the POEMS syndrome. The reported cases of this syndrome among Thais are summarized.
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PMID:POEMS syndrome: a case with proliferative vasculopathy and a review of cases Thailand. 796 31

We present a case of an unusual multi-systematic disorder whose cardinal signs were severe progressive sensorimotor polyneuropathy, hepatomegaly, endocrinopathy, plasma cell dyscrasia with osteosclerotic bone lesions and M-protein production as well as skin lesions (termed POEMS Syndrome) whose pathophysiology is still unknown, as well as its relationship with myeloma multiplex. The patient, 67 years old, had a history of progressive weakness and numbness of the lower legs, clinically revealed as sensomotor polyneuropathy, hepatomegaly, IgG lambda monoclonal protein in the serum, endocrine abnormalities and skin lesions. The final and definite diagnosis was established by open, surgical biopsy of the second lumbal vertebra.
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PMID:[Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes: the POEMS syndrome]. 820 22

POEMS syndrome is a systemic disorder with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. The association of POEMS syndrome with lympho-proliferative disorder is very commun. The pathogenesis remains poorly understood but implication of cytokines (interleukins 1 and 6) is suspected. We report a case of a classic POEMS syndrome (with polyneuropathy, hepatomegaly, diabetes melitus, hyperpigmentation, monoclonal IgG lambda, anasarca and solitary plasmocytoma), associated with high serum levels of interleukin 6.
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PMID:[POEMS syndrome: role and value of interleukin-6]. 878 86

We studied pathogenesis of hepatomegaly in Crow-Fukase syndrome. Hepatocytes were isolated from BALB/c mice and cultured with serum or ascites of three patients with Crow-Fukase syndrome. After 24 hours culture, total number of hepatocytes was counted. The levels of human hepatocyte growth factor (h-HGF) in the serum were also measured. In the hepatocyte culture study, the numbers of hepatocytes after 24 hours cultured in the serum of two patients were significantly larger than those of the control group. When the hepatocytes were cultured with patients' serum after corticosteroid therapy, the survival numbers were markedly reduced. The serum levels of h-HGF in our three patients were 0.62, 0.52, 0.53 ng/ml respectively. They were all slightly higher than normal values (< or = 0.39 ng/ml). Our study indicates that in the serum or ascites of patients with Crow-Fukase syndrome, there may be some humoral factors other than h-HGF, which promote proliferation of hepatocyte or protect hepatocyte.
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PMID:[A study on the pathogenesis of hepatomegaly in patients with Crow-Fukase syndrome]. 881 Aug 45

We reported a case of Crow-Fukase syndrome and examined the mechanism of hepatomegaly in Crow-Fukase syndrome. A 67-year-old woman was presented with polyneruopathy, hepatosplenomegaly, pericardial effusion and M proteinemia. On examination, all laboratory data and clinical symptoms were compatible with Crow-Fukase syndrome. The patient was treated with prednisolone and immunoadsorption plasmaphresis therapy. All the clinical manifestations including hepatomegaly gradually improved. In order to examine whether some factors that promote the prolifelation of hepatocyte may exist in the patient's serum, we cultured mouse hepatocyte in the presence of patient's or control serum together. Though the number of hepatocytes decreased after 2 days culture, the number of hepatocytes cultured with patient's serum in active disease stage remained significantly greater than of hepatocytes cultured with either control serum or patient's serum in healing stage. There were no abnormal pathological findings in biopsied liver. Liver suggesting that hepatomegaly was the results of normal hepatocytes proliferation. Taken together, these findings suggest there were some factors that may promote the proliferation of hepatocytes or may have protective effect of hepatocyte in patient's serum. Though the level of human hepatocyte growth factor (h-HGF) in patient's serum in active disease stage was slightly increased, hepatomegaly cannot be attributable solely to h-HGF. Organomegaly is one of the important symptoms of the Crow-Fukase syndrome, however, in so far as we are aware its mechanism is not examined. In this report it is suggested that several unknown factors other than h-HGF may contribute the hepatomegaly of the Crow-Fukase syndrome.
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PMID:[The detection of factors that may promote the hepatocyte proliferation in the serum of a patient with crow-fukase syndrome]. 885 11


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