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

A case of plasma cell dyscrasia with polyneuropathy and endocrine disorder is reported. Clinically, polycythemia vera, gynecomastia, pigmentation of the skin, hepatosplenomegaly, renal enlargement and severe polyneuropathy in the lower extremities were recognized. The peculiarity of this case was polycythemia vera that had been present for several years before manifestation of the clinical symptoms. Microscopically, retroperitoneal lymph nodes showed angio-follicular lymphoid hyperplasia and plasma cell infiltration in the interfollicular region. By means of the avidin-biotin-peroxidase complex method, plasma cells were positive for lambda light chain, IgA and IgG. Severe segmental demyelination and slight axonal atrophy were found in a sural nerve biopsy.
 ...
PMID:Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome, associated with preceding polycythemia vera. A case report and review of the literature. 306 44

Four middle-aged male Chinese with polyneuropathy, skin hyperpigmentation, oedema, hepatosplenomegaly, ascites, gynaecomastia and white nails are described. In Japan and United States this syndrome has been associated with plasma cell dyscrasia. However, neither M-protein nor skeletal lesions were demonstrated in these four patients.
 ...
PMID:Syndrome of polyneuropathy, skin hyperpigmentation, oedema and hepatosplenomegaly. 666 9

Clinical analysis of 293 cases of cirrhosis from two moderate sized hospitals in the city of Dacca has been presented. Maximum number of cases were in the age group over 40 with 150 (51.2%) males and 19 (5.8%) females. Significant past history included viral hepatitis (21.5%), kala-azar (11.6%) and malaria (10.24%). History of alcoholism was present only in 16 (5.5%) cases. Weakness (84.3%), weight loss (72%) and anorexia (39.3%) constituted the most common symptoms. Ascites (45%), haematemesis (11.6%) and melaena (28.7%) were the next common symptoms. Hepatosplenomegaly was found in about one-third of the cases. Testicular atrophy was recorded in 41.63% cases whereas gynaecomastia was relatively less common (5.5%). Scanty body hair and white nails were present in almost equal number of cases (14.7% and 18%). The cases presented here are those with overt manifestation. Nevertheless, the clinical features are not materially different from those reported by other authors. In the absence of alcoholism, viral hepatitis is presumably the most important aetiological factor in our cases and the clinical features compare favourably with non-alcoholic cirrhosis of the western writers. Cryptogenic cirrhosis has been considered to be most common type constituting 43.7% of our cases.
 ...
PMID:Cirrhosis of liver. 734 4

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.
 ...
PMID:Plasma cell dyscrasia with polyneuritis and dermato-endocrine alterations. Report of a new case outside Japan. 741 45

A 30 years old, non-alcoholic farmer from Trishal, Mymensingh was admitted in Mymensingh Medical College Hospital on 7 February, 2004 with the complaints of gradual swelling of abdomen, both legs and upper abdominal pain for 3 months. For the last 6 years, he was treated as a case of chronic liver disease (CLD) with spironolactone and frusemide. He was non-icteric, mildly anaemic with mild oedema, clubbing, gynaecomastia and engorged vein over anterior abdominal wall, flanks and back. Direction of venous flow was from below upward. There were mild hepatosplenomegaly, ascites and bilateral testicular atrophy. He was diagnosed as a case of Budd-Chiari Syndrome (BCS) on the basis of physical examination and it was confirmed by the findings of ultrasonography, liver scan and doppler study. The patient was managed by medical therapy alone.
 ...
PMID:Budd-Chiari syndrome. 1569 63

POEMS syndrome is a rare plasma cell dyscrasia which is characterized by small amounts of monoclonal protein, and a multisystem complex manifested by various combinations of polyneuropathy, organomegaly, endocrinopathy and skin changes. Here, we presented an atypical case of POEMS syndrome with IgG kappa monoclonal protein, chronic demyelinating polyneuropathy, hepatosplenomegaly, hypothyroidism, gynecomastia and severe renal impairment. The finding of IgG kappa type of monoclonal protein in our patient was interesting because the majority of cases were reported to have lambda light chain. Also, the absence of typical skin and bone lesions were atypical. Though speculative, these atypical features may account for the unusual presentation of this case. Our patient rapidly progressed to end-stage renal failure and died of cachexia. Renal involvement in POEMS syndrome is rare but may show substantial clinical and pathological variations. Proteinuria, hematuria, renal dysfunction and renal failure requiring hemodialysis can be seen. The pathogenesis of renal dysfunction is unclear. As a conclusion, POEMS syndrome may present with diverse clinicopathologic manifestations. In this syndrome, renal involvement may lead to end stage renal failure and the course may be fatal due to severe polyneuropathy and wasting.
 ...
PMID:An atypical case of POEMS syndrome with IgG kappa M protein and end stage renal failure. 1630 46