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)

Primary renal lymphoma is a rare disease. There is still no agreement on its development primarily in this non-lymphoid organ. Some of the reported cases are questionable because of the incomplete staging of extrarenal disease. In the present study, we describe a rare case of a 73-year-old white man presenting with right flank pain. Physical examination did not reveal any peripheral lymphadenopathy or hepatosplenomegaly. An abdominal computerized tomography (CT) showed a large tumor in the right kidney with no enlarged liver, spleen or lymph nodes. On morphological and immunohistochemical examination, the tumor fulfilled the criteria of anaplastic large cell lymphoma (ALCL). A review of the literature revealed that this is the first reported case of primary ALCL of the kidney.
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PMID:Primary anaplastic large cell lymphoma of the kidney. 1268 57

We report the case of a 64-year-old male in whom fever, cutaneous rash and hepatomegaly were the first manifestations of Hodgkin disease (HD). Histologically a dense lymphohistiocytic infiltrate with a granulomatous pattern was found in the skin infiltrate. A computed tomography scan revealed hepatosplenomegaly and a small retroperitoneal lymphadenopathy. An hepatic percutaneous biopsy showed a granulomatous infiltration with typical Reed-Sternberg cells. Cutaneous manifestations of HD are briefly reviewed. The authors underline that granulomatous infiltration of the skin as the first manifestation of lymphoma is a very rare feature. We also discuss the possible pathogenic mechanisms of skin granulomas.
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PMID:Cutaneous granulomas as the first manifestation of Hodgkin's disease. 1280 95

The literature was reviewed to study cases of intoxication with systemic dermatitis associated with exposure to trichloroethylene. The average age of patients in the reports reviewed to date was twenty-nine; these diseases were found in relatively young persons and no difference was found according to gender. Many cases occurred within one month after the onset of exposure to trichloroethylene, and were accompanied by hepatitis, jaundice, hepatomegaly or hepatosplenomegaly. Most of the patients had no history of drug abuse or herpes infection. The level of exposure to trichloroethylene was not recorded in many cases, but ranged from less than 9 ppm to 800 ppm. In the severest cases, the lesions involved mucous membranes such as the conjunctiva and oral cavity, and the patients were diagnosed with Stevens-Johnson syndrome, but the etiology of the disease after trichloroethylene exposure remains unclear. Since several drugs have also been shown to cause systemic dermatitis with hepatitis, susceptibility factors are discussed. Many patients were found to have the slow acetylator genotype of N-acetyltransferase (NAT) 2, suggesting that the NAT2 genotype is a susceptibility factor. This hypothesis may also be applicable to trichloroethylene because NAT is involved in the glutathione-mediated metabolism.
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PMID:Generalized skin reactions in relation to trichloroethylene exposure: a review from the viewpoint of drug-metabolizing enzymes. 1460 23

The prognostic significance of immunophenotypical properties of leukaemic cells is well known. However, the biological and clinical significance of CD7 and CD56 antigen expression in acute leukaemias are not clearly established. In patients with acute leukaemias, we identified CD7 and CD56 expression and analysed their associations with markers expressed early in haemopoietic ontogeny and clinical parameters. Among 22 patients with acute leukaemia [12 acute myeloblastic leukaemia (AML), 10 acute lymphoblastic leukaemia (ALL)], we found CD7 positivity in 15 of 22 patients (68%) and CD56 positivity in four patients (18%). CD7 positivity was observed in seven patients (58%) with AML and in eight patients (80%) with ALL. CD56 positivity was observed in three patients (25%) with AML and one patient (10%) with ALL. Lymphadenopathy was present in five patients and associated with hepatosplenomegaly in three patients with ALL. Splenomegaly and hepatomegaly were present in three patients with AML. Central nervous system involvement was seen in one patient with ALL. Complete remission was achieved in nine patients (41%) (five ALL and four AML). Our data showed that CD7 and CD56 positivity at diagnosis associated with low remission rate and biological aggressiveness in a significant proportion of patients. We suggest the evaluation of CD7 and CD56 in all patients with acute leukaemias at the time of diagnosis in view of poor clinical outcome.
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PMID:The importance of CD7 and CD56 antigens in acute leukaemias. 1505 63

Hepatosplenic schistosomiasis involving organomegaly, portal fibrosis and portal hypertension has been observed in autopsy studies. Here, we have tested the hypothesis that hepatosplenic disease including organomegaly and markers of increased portal pressure can occur in school aged children in the absence of fibrosis. A case-only study of 96 children aged 7-20 years defined by ultrasound detectable hepatomegaly was undertaken in Makueni district, Kenya. A novel method of clinical examination that involved a consensus scoring by three or four examiners was used to classify children as presenting with severe or moderate hepatosplenic disease after palpation of livers and spleens. Ultrasound examination of livers and spleens was based on the Niamey protocol. Clinical measurements included spleen enlargement along the mid-clavicular and mid-axillary lines, liver enlargement along the mid-sternal (MSL) and mid-clavicular lines, as well as organ consistency. The clinical examination indicated that 9% and 60% of the children had severe or moderate hepatosplenomegaly, respectively. Amongst egg-positive children, all clinical measurements, except MSL liver enlargement, correlated with egg count, as did portal vein diameter, spleen length and liver length measured by ultrasound. Peri-portal fibrosis was not observed in any child, whereas 28% of the children were classified as having increased portal pressure according to World Health Organization criteria. There was no effect of malaria parasitaemia or hepatitis seropositvity on any of the observed parameters. These results indicate that hepatosplenic disease in school-aged children attributable to S. mansoni infection, involving hepatosplenomegaly and increased portal vein diameter, can occur in the absence of peri-portal fibrosis.
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PMID:Detailed clinical and ultrasound examination of children and adolescents in a Schistosoma mansoni endemic area in Kenya: hepatosplenic disease in the absence of portal fibrosis. 1507 64

A 40 year old man presented with abdominal pain, jaundice, weight loss, and hepatosplenomegaly. Liver function tests revealed cholestatic jaundice and a computed tomography scan showed an enlarged liver, with a normal biliary tree. Liver biopsy showed diffuse infiltration by neutrophils, monocytoid cells, and blasts. Peripheral blood film and bone marrow were consistent with acute myeloid leukaemia. After treatment with chemotherapy using an acute myeloid leukaemia protocol (UK Medical Research Council AML-12), there was complete resolution of jaundice and the patient went into complete molecular remission.
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PMID:Acute myeloid leukaemia presenting as cholestatic hepatitis. 1511 66

We present three cases of transient abnormal myelopoiesis associated with trisomy 21 in which hepatomegaly was apparent during the fetal period. In the first case, the fetal hepatosplenomegaly was severe, multiple organ failure occurred in the neonatal period and death ensued at 4 weeks of age. In the second case, the hepatomegaly was moderate, and with conservative treatment in the neonatal period the outcome was good. In the third case, hepatomegaly was mild and improved spontaneously, and the hematological abnormalities required only conservative treatment in the neonatal period. Our experience raises the question of whether fetal hepatosplenomegaly is a predictor of transient myeloproliferative disorder with trisomy 21 and whether the degree of fetal hepatomegaly is a marker for the neonatal severity of hematological abnormalities.
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PMID:Is the degree of fetal hepatosplenomegaly with transient abnormal myelopoiesis closely related to the postnatal severity of hematological abnormalities in Down syndrome? 1522 21

Thrombocytopenic purpura associated with brucellosis has been rarely reported in the world literature. Thrombocytopenic purpura is generally part of the array of manifestations of brucellosis such as fever, arthritis, malaise and hepatosplenomegaly. We describe a nine-year-old girl who presented with thrombocytopenic purpura as the sole manifestation of brucellosis, which resolved with anti-Brucella chemotherapy. Her physical examination was remarkable for hepatomegaly of 3 cm and splenomegaly of 2 cm palpable below the costal margin. Initial laboratory investigations revealed isolated thrombocytopenia with platelet count of 11,300/mm3 and positive serology for Brucella. Thrombocytopenia resolved promptly with proper antibiotics on 7th day of treatment. Brucellosis should be included in the differential diagnosis of thrombocytopenic purpura in Brucella-endemic areas.
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PMID:Thrombocytopenic purpura as only manifestation of brucellosis in a child. 1550 83

Evaluating regression of morbidity associated with parasitic infections is an important component of community-based control programmes. We performed an intervention against Schistosoma mansoni infection, focusing on hepatosplenomegaly in the absence of periportal fibrosis, in a cohort of 67 Kenyan children aged 7-18 years from Makueni District, selected on the basis of hepatosplenomegaly detected by ultrasonography. Clinical and ultrasound examinations were conducted annually for three years after treatment, and the source of infection (a river) was regularly treated with molluscicide, thereby severely reducing exposure to schistosomiasis. Malaria transmission was uninterrupted. The prevalence of hard spleens, and the magnitude of clinically assessed splenomegaly along the mid-axillary and mid-clavicular lines decreased monotonically over time, independently of age, whereas clinically measured hepatomegaly along the mid-sternal line and the prevalence of firm livers decreased in an age-specific manner, being more pronounced amongst children aged 14 years or older at enrolment. Ultrasound data were less informative, and did not concur with clinical observations. These results demonstrate that praziquantel treatment reduces hepatosplenomegaly in the absence of exposure to S. mansoni, even with continuing exposure to malaria. The lack of complete resolution of hepatosplenomegaly in most children suggests, among other things, a residual organomegaly attributable to malaria.
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PMID:Regression of hepatosplenomegaly in Kenyan school-aged children after praziquantel treatment and three years of greatly reduced exposure to Schistosoma mansoni. 1560 41

Systemic form of juvenile xanthogranuloma with involvement of liver and bone marrow is reported in a 2-month-old female infant who presented with hepatosplenomegaly, severe anemia, and thrombocytopenia. There was no skin lesion, nor bone lesion. The enlarged liver has generalized yellowish spots. The diagnosis of juvenile xanthogranuloma was made by pathologic findings of marrow and portal tract infiltration by S-100 negative, CD1a negative, CD68 positive, and Factor XIIIa positive large pale to foamy histiocytes with Touton giant cells, and lack of Langerhans cell granule by electron microscopic examination. The patient was treated with Vinblastine and Etoposide, and experienced slow and gradual disease regression in one year. To the best of knowledge, this is the first documented case of bone marrow involvement in systemic juvenile xanthogranuloma.
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PMID:Systemic form of juvenile xanthogranuloma: report of a case with liver and bone marrow involvement. 1563 May 37


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