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 72-year-old male had complained of right back pain and bleeding from his tongue. He was admitted to our department on May 18, 1989. Physical examination revealed hepatosplenomegaly. Peripheral blood findings were as follows: RBC was 3.80 x 10(6)/microliters. Hb 12.2 g/dl, Ht 36.5%, platelet count 735 x 10(3)/microliters, WBC 22,100/microliters, leukoerythroblastosis present. Neutrophil alkaline phosphatase score was normal. Serum vitamin B12 and plasma platelet-derived growth factor level were elevated. Skeletal X-ray revealed multiple punched-out lesions at the 8th thoracic vertebra, and 6th and 8th ribs. Serum IgG level was 3,900 mg/dl. Serum immunoelectrophoresis revealed IgG lambda-type M-protein. Because he complained of severe cervical pain, and skeletal X-ray examination revealed the fracture of 6th cervical vertebra, the operation was performed to remove the lesion. Biopsy of cervical lesion revealed plasmacytoma. M-protein was decreased and the size of the tumor was reduced after treatment with VCAP (vincristine, cyclophosphamide, adriamycin, prednisolone) regimen and interferon-alpha for multiple myeloma.
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PMID:[IgG lambda-type multiple myeloma associated with myelofibrosis accompanied by thrombocytosis]. 194 35

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

The temporal susceptibility of tumors to induction of necrosis and regression by endotoxin was investigated further with a focus on the role of the putative mediator, tumor necrosis factor (TNF). Production of this factor was shown earlier to require prior activation of the mononuclear phagocytic system (MPS). Transplants of Meth A sarcoma or MOPC315 plasmacytoma had no consistent effect on parameters of MPS function such as hepatosplenomegaly, carbon clearance and non-specific antibacterial resistance at times that they were sensitive to induction of necrosis. Moreover, TNF, quantified by its necrotizing and regressing activity in vivo, could not be detected in the serum of tumor hosts after a necrotizing dose of endotoxin, while much smaller volumes of serum with TNF (TNS) of appropriately treated donor mice showed activity. As repeated incubation of TNS with Meth A cells at 37 degrees C hardly removed its in vivo activity against Meth A, immediate absorption of produced TNF to the tumor cell mass seems a less likely cause. Cytostatic activity, another property attributed to TNF, was hardly increased in post-endotoxin tumor host serum, while TNS is highly cytostatic. It is concluded that induction of tumor necrosis is not dependent on MPS activation. A role of TNF as mediator of the effects of endotoxin still remains uncertain. Furthermore, the present and other data suggest that TNF, like endotoxin, probably acts by an indirect mechanism against tumors in vivo.
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PMID:Role of mononuclear phagocyte function in endotoxin-induced tumor necrosis. 636 47

A panel of 11 IgG monoclonal antierythrocyte antibodies was generated by fusing spleen and bone marrow cells from unimmunized New Zealand black mice with the nonsecreting murine plasmacytoma cell line P3.X63.NS1. The monoclonal antibodies were detected by indirect hemagglutination of unaltered erythrocytes from several strains of mice. Seven of the antibodies cross-reacted with rat erythrocytes, but none of the antibodies agglutinated erythrocytes from any other species tested. Seven of the monoclonal antibodies were also capable of fixing rabbit complement. In vivo studies utilizing these 11 IgG-secreting hybridomas were performed in syngeneic BALB/c mice. Mice injected with nine of the hybridomas showed positive direct antiglobulin test results but did not become anemic. In contrast, hybridoma 114, secreting an IgG3 antibody, and hybridoma 245, secreting an IgG1 antibody, were both capable of mediating an acute, rapidly fatal hemolytic anemia. Intraperitoneal injection of hybridomas 114 and 245 resulted in positive direct and indirect antiglobulin test results, decreased hematocrit level, and reticulocytosis 3 to 6 days after cell injection. The mice survived a mean of 8 days, and death was associated with severe anemia and spontaneous erythrocyte agglutination. Autopsy studies revealed hepatosplenomegaly, small mesenteric tumor (hybridoma) mass, and no ascites. The liver and spleens were characterized histologically by erythrophagocytosis, extramedullary hematopoiesis, and hemosiderin deposition. Acute hemolytic anemia in BALB/c mice mediated by hybridomas 114 and 245 represents a new animal model that can be used to further define the mechanisms of immune hemolytic disease.
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PMID:Monoclonal antibody-induced murine hemolytic anemia. 648 Dec 19

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

A new syndrome has been described comprising polyneuropathy, oedema, hyperpigmentation and thickening of the skin, gynaecomastia in males and amenorrhoea in females, monoclonal gammopathy, papilloedema and diabetes. There is frequent osteosclerosis with or without plasmacytoma, hepatosplenomegaly and polycythaemia. There is a good response to corticosteroids, immunosuppressive drugs and occasionally to excision or irradiation of the plasmacytoma. This syndrome was first described in Japan, and is still seen predominantly there, and only occasionally in other areas. An example in a 51-year-old Spanish female is described: she had a spectacular response to prednisone and melphalan. The aetiopathogenic possibilities are discussed.
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PMID:Plasma cell dyscrasia with polyneuritis and dermato-endocrine alterations. Report of a new case outside Japan. 741 45

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.
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PMID:POEMS syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma. 1032 21

A 57-year-old woman suffering from pleural and pericardial effusion, pulmonary hypertention, lymphadenopathy, hepatosplenomegaly, edema, hypertrichosis, small hemangioma and polyneuropathy was diagnosed as Crow-Fukase syndrome. Osteoctomy of the left second rib and irradiation of this rib and the left iliac bone were performed. Serum vascular endothelial growth factor (VEGF) level decreased to less than one-half the level before the operation (from 5,180 to 2,150 pg/ml). Immediately after the operation, pleural and pericardial effusions due to hyperpenetration improved, and polyneuropathy and hypertrichosis due to hypervasularity also gradually improved. The resected lesion was histopathologically found to be of a plasmacytoma of the IgG lambda type. Since the level of VEGF in the tissue specimen was much lower (116 pg/ml) than that in the serum, VEGF could not have been produced by the plasmacytoma.
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PMID:[A case of Crow-Fukase syndrome showing improvement following excision and irradiation of bone lesions]. 1529 61

Total 14 cases of myeloma in young age group (<40 years) have been reported out of 178 cases of myeloma in a time period of 7 years (1993-1999). Males predominated overfe males. Like adult myeloma, patients presented mostly with the backache, pain in pelvis, lower spine and weakness in about 60% of cases followed by swelling of bone in 40% of cases. One case presented with bleeding gum, malena and hepatosplenomegaly and was diagnosed as plasma cell leukemia. Radiological examination revealed lytic lesion in almost all the cases with fracture femur and rib in 28.57% of cases. Anaemia and raised ESR was noted in all the cases. Myeloma typing revealed IgG myeloma in 10 cases, light chain myeloma in 3 cases and IgA myeloma in one case. None of the patient was traceable after 2 years. Thus our study concludes that myeloma in the young age in India occurs in increased frequency and clinically presents just like adult and elderly myeloma, but serologically are predominantly of IgG type. There is also an increased frequency of solitary plasmacytoma as compared to adult myeloma.
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PMID:Myeloma in young age. 1676 40


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