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)

Multiple myeloma associated primary biliary cirrhosis (PBC) is very rare and only two cases have been reported. In this paper, we reported the first case of male patient with asymptomatic PBC and multiple myeloma. A 66 year-old Japanese male was referred to our hospital for the further examination of a monoclonal gammopathy. He was diagnosed of multiple myeloma (IgG-lambda type) because of 2.3 g/day of Bence Jones proteinuria (lambda type), 3,401 mg/dl of monoclonal IgG (lambda type), 12.8% of bone marrow plasmocytosis and generalized osteoporosis. The alkaline phosphatase was 314 mU/ml and serum IgM level (polyclonal) 937 mg/dl. The patient was started on intermittent courses of melphalan and prednisolone, achieving transient improvement. After two years, hepatosplenomegaly developed gradually and the levels of serum ALP elevated increasingly. At that time, relevant investigation results were: serum ALP 663 mU/ml, serum IgG 4,144 mg/dl, serum IgM 823 mg/dl, positive anti-mitochondrial antibody test x 320. The liver biopsy showed chronic nonsuppurative destructive cholangitis. PBC (stage 1-2 according to Sheuer's criteria) associated with multiple myeloma was diagnosed. A pathogenetic relationship such as loss of immunoregulatory function could be speculated although the simultaneous occurrence of PBC and multiple myeloma could be coincidental.
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PMID:[Multiple myeloma of IgG-lambda type associated with asymptomatic primary biliary cirrhosis]. 251 98

We experienced a double infection of tuberculosis and amebiasis of the liver. A 28 year old male with AIDS was admitted to our hospital because of severe diarrhea and liver abscess by Entamoeba histolytica. In spite of improvement of the diarrhea and liver abscess by the therapy against E. historicica, serum levels of gamma-GTP and ALP remained high and hepatosplenomegaly gradually increased. A liver biopsy was performed. Pathology showed a granulomatous lesion with Langhans' giant cells. From this specimen, IS6110 gene, a specific DNA for Mycobacterium tuberculosis was detected by PCR method. After anti-tuberculosis treatment was given for 6 months the increased serum gamma-GTP, ALP decreased and hepatosplenomegaly diminished.
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PMID:[A case of AIDS complicated with liver tuberculosis]. 1114 84

This study was undertaken to assess the biochemical changes induced in chronic schistosomiasis and/or chronic HCV, as well as to pinpoint the most significant parameters which could be used as dependable indices for the differentiation of single and coupled infections with or without liver cirrhosis. The selected patients were allocated into 2 broad groups: GrII (Schistosomiasis) which was subdivided into 3 subgroups: GrII(a) schistosomal patients with hepatosplenomegaly; GrII(b) hepatosplenic schistosomal patients with decompensated liver cirrhosis; GrII(c) schistosomal patients with no organomegaly. GrIII (Combined) comprised 2 subgroups: GrIII(a) schistosomal-HCV infection with decompensated liver cirrhosis; GrIII(b) schistosomal-HCV infection without liver cirrhosis. For statistical comparison normal healthy subjects were taken as a reference group (Gr I). Results showed that schistosomal patients without organomegaly manifested non significant changes in all studied parameters compared to normal controls. Highly significant elevations in serum ALT, AST, ALP and GGT activities were recorded in all other subgroups but the highest levels are reported in GrIIb. AST/ALT and direct/indirect bilirubin ratios were highest in GrIIIa (1.17+/-0.26, 1.54 +/- 0.37, respectively). Serum total protein and albumin levels showed the highest reduction (33 and 59%) concomitantly with the highest increase in gamma-globulin level (75%) in GrIII(a). Blood total iron was significantly reduced in GrII(a,b) (15.6 and 12%) (8.8%) bilirubin, GGT and AST in this order are good discriminators between the different subgroups in GrII. On the other hand, ALT, AST, albumin, ALP, GGT, protein and direct bilirubin are the most significant indices to differentiate chronic schistosomiasis and the combined group with/or without liver cirrhosis.
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PMID:Biochemical changes in patients with combined chronic schistosomiasis and viral hepatitis C infections. 1138 Nov 90

A 68-year-old man was admitted to our hospital because of fever, jaundice and hepatosplenomegaly. A diagnosis of diffuse large cell, B-cell type malignant lymphoma, associated with hemophagocytic syndrome (LAHS), was made. CT scan revealed lymphadenopathy in the abdominal cavity and multiple tumors in the spleen. Performance status and hepatic coma grade were 4 and II, respectively. Laboratory findings showed bicytopenia (Hb 9.9 g/dl, platelet 35 x 10(3)/microliter), severe liver dysfunction (ALP 1,115 U/l, gamma-GTP 437 U/l, T.Bil 15.4 mg/dl, D.Bil 12.8 mg/dl) and elevated levels of beta 2 microglobulin (12.9 mg/dl), ferritin (2,300 ng/ml) and sIL-2 receptor (36,900 U/ml). Plasma exchange (PE) and continuous hemodiafiltration (CHDF) enabled the patient to undergo diagnostic procedures, irradiation (total 34 Gy) and chemotherapy. Biopsy specimens revealed infiltration of lymphoma cells into the liver and bone marrow. We measured the blood concentrations of TNF-alpha, IL-6, and IL-8 before and after PE and CHDF by the ELISA method, and found normalization of hypercytokinemia after the procedure. It was suggested that initial treatment with PE and CHDF was effective for control of HPS, enabling us to perform chemotherapy for the lymphoma.
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PMID:[Plasma exchange and continuous hemodiafiltration as an initial treatment for diffuse large B-cell lymphoma-associated hemophagocytic syndrome]. 1186 63

Although liver involvement is frequently seen in brucellosis, acute hepatitis is a rare clinical entity. In its progress, haematological findings are non-specific and vary in respect to severity. In this paper, we present a case of brucellosis with acute hepatitis and bicytopenia without anaemia. A 19-year-old man presented with a 2-week history of fever, sweating, low back and leg pain, lassitude, loss appetite, nausea and vomiting. He gave a history of raw milk ingestion and animal contact. Physical examination showed signs of icteric skin and sclera, tenderness in the right hypochondriac region and hepatosplenomegaly. On admission to hospital, laboratory tests showed WBC 3500/mmc (polymorphs 63% and lymphocytes 33%), haemoglobin 13.8 g/dL, platelet 89000/mmc, erythrocyte sedimentation rate 19 mm/h, and C-reactive protein 21.7 mg/dL (N<0.8 mg/dL). Biochemical tests were as follows: AST 771 U/L, ALT 471 U/L, ALP 355 U/L, GGT 432 U/L, total bilirubin 2.61 mg/dL, direct bilirubin 1.45 mg/dL and albumin 3.7 g/dL. Viral hepatitis markers were found to be negative (HBsAg, anti-HBc total, anti-HBc IgM, anti-HAV IgM, and anti-HCV). Blood culture grew Brucella melitensis. Leukopenia and thrombocytopenia returned to normal levels at the 7th and 14th day of his admission, respectively. Liver function tests improved at the 28th day. Treatment of the brucellosis was performed with antibiotics (tetracycline 500 mg orally four times daily for 6 weeks and streptomycin 1 g IM once daily for 21 days). Finally, a case of brucellosis with acute hepatitis and bicytopenia was treated with a successful outcome. In conclusion, we suggest that due consideration be taken of bicytopenia/pancytopenia and acute hepatitis in brucellosis cases in Turkey, an endemic region.
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PMID:A case of brucellosis presenting with acute hepatitis and bicytopenia. 2611 Mar

A 76-year-old woman was referred to our hospital because of fever, hemorrhagic skin lesion with pruritus, and severe thrombocytopenia. Anemia; marked eosinophilia; and elevated ALP, CRP, and soluble IL-2 receptor levels were observed on admission. Both anti-nuclear antibody and Coombs tests were positive. Computed tomography revealed bilateral pleural effusion, ascites, abdominal lymphadenopathy, and mild hepatosplenomegaly. A thorough examination for the initial differential diagnoses excluded the possibility of myeloid/lymphoid neoplasms with eosinophilia and gene rearrangement, infectious diseases, and eosinophilic granulomatosis with polyangiitis. Remaining possibilities included angioimmunoblastic T-cell lymphoma (AITL) and systemic inflammatory disorders. Although AITL was plausible, there was no histological evidence to support the diagnosis. The patient was then administered prednisolone alone, which led to a lasting resolution of her symptoms. The atypical AITL course raised the suspicion of a misdiagnosis; thus the possibility of an inflammatory disease was reconsidered. TAFRO syndrome was suspected owing to its characteristic clinical features (thrombocytopenia, anasarca, fever and organomegaly). Since a definitive diagnosis required the exclusion of systemic lupus erythematosus (SLE), anti-double-stranded DNA antibody was tested in the initial frozen serum sample. An unexpected positive result led to the final diagnosis of SLE. Here, we report a rare case of SLE lacking typical symptoms and exhibiting various hematological abnormalities, such as eosinophilia.
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PMID:[Systemic lupus erythematosus with marked eosinophilia and clinical features mimicking TAFRO syndrome]. 2997 44