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)

Systemic candidiasis with Candida-induced abscesses, predominantly in the liver and the spleen, was diagnosed in 27 patients with haematologic malignancies after intensive cytostatic therapy. Specific features included septic fever unresponsive to antimicrobial therapy, hepatosplenomegaly with multiple lesions in the liver and spleen (diameter up to 2 cm) as detected by computed tomography (CT) or ultrasound, and an elevation in liver enzymes. During treatment, induced neutropenia, hepatic and splenic foci were poorly defined histologically and were not identified by imaging procedures. After granulocyte recovery these foci showed characteristic histological patterns. Ultrasound and/or CT investigations of the abdomen now revealed characteristic lesions in the liver and the spleen. Gamma-GT and alkaline phosphatase were early indicators of hepatic involvement in Candida septicaemia and were often already elevated in aplasia.
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PMID:Hepatosplenic candidiasis, a late manifestation of Candida septicaemia in neutropenic patients with haematologic malignancies. 233 85

In 40 patients (17 male, 23 female, median age 57 years) with the presumptive diagnosis of primary (essential) thrombocythemia (PTH) according to the diagnostic requirements of the Polycythemia-Vera-Study-Group (PVSG) a follow-up study and a histological evaluation of initial trephine biopsies of the bone marrow were performed. Thorough review of the hematological data during the lengthy course of disease (observation time ranging from 1.5-10.5 years) and the histomorphology of the bone marrow implied a discrimination into two groups of patients. Group I patients (n = 26; 10 male, 16 female) were compatible with PTH according to our follow-up studies. Group II patients consisted of 14 cases (7 male, 7 female) which suggested retrospectively early hyperplastic stages of agnogenic myeloid metaplasia (AMM) with concomitant thrombocytosis. In PTH (group I patients) there was a sustained elevation of the platelet count lasting for several years accompanied by stable other blood values. Early AMM (group II patients) was characterized by an insidious decline of the initially elevated thrombocyte count, starting in a few patients already 4-6 months after admission. In AMM there was further an increase in hepatosplenomegaly observable together with the level of LDH and the score of the leukocyte alkaline phosphatase, and finally an evolution of a leukoerythroblastic blood picture could be noticed. Initial histopathology of the bone marrow revealed a profound proliferation of a not severely dysplastic megakaryopoiesis in group I patients (PTH) and a normal content of reticulin fibers. In early thrombocythemic AMM (group II patients) conspicuous abnormalities of megakaryocytes were accompanied by a slight to moderate increase in argyrophilic fibers and a left-shifted neutrophilic granulocyto- as well as erythropoiesis. These differences of certain histomorphological features could be substantiated by morphometric analysis. Our findings suggest that even the rigid requirements for the diagnosis of PTH as proposed by the PVSG may not be sufficiently restrictive to exclude patients with early hyperplastic stages of thrombocythemic AMM.
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PMID:Primary (essential) thrombocythemia versus initial (hyperplastic) stages of agnogenic myeloid metaplasia with thrombocytosis--a critical evaluation of clinical and histomorphological data. 247 28

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

Autoimmune chronic active hepatitis (ACAH) is an infrequent modality of chronic hepatitis (CH) with clinical and laboratory findings of an autoimmune disorder. Clinical and pathological findings of 7 cases are presented; all were females with ages between 7 and 24 years. Main symptoms and signs were weight loss, malaise, arthralgias, fever, menstrual disturbances, hepatosplenomegaly, jaundice ascites and esophageal varices. Aminotransferases were elevated in all cases, and bilirubin, alkaline phosphatase and gammaglobulins were found to be raised in six. Antinuclear antibodies were positive in 5 cases, smooth muscle antibodies in 3, and antimitochondrial antibodies were detected in one. Morphological changes were those of chronic active hepatitis with variable degrees of fibrosis. Plasma cells were conspicuous. All patients received steroid treatment (Prednisone). It is concluded that the diagnosis of ACAH can be based on clinical and immunological criteria provided other causes of CH such as viral hepatitis, are ruled out.
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PMID:[Autoimmune chronic hepatitis: clinico-pathologic spectrum in 7 cases]. 251 21

Chronic neutrophilic leukemia (CNL) is a rare type of leukemia. We diagnosed a 81-year-old woman as CNL because she showed that sustained leukocytosis dominated by mature neutrophils, hepatosplenomegaly, high neutrophilic alkaline phosphatase (NAP) score, absence of the Ph1 chromosome and no evidence of leukemoid reaction. During the clinical course, she did not manifest hemorrhagic tendency or infection. We also examined neutrophilic function including chemotaxis, chemiluminescence, nitroblue tetrazolium (NBT) dye reduction, which all indicated normal neutrophil function. Using a reversed phase-high pressure liquid chromatography (HPLC), we detected the production of leukotriene B4 (LTB4) in neutrophils. We found that the LTB4 production was decreased in neutrophils whereas they showed normal chemotaxis. This discrepancy has never, to our knowledge, been reported before in case of CNL.
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PMID:The discrepancy between chemotaxis and leukotriene B4 production in a patient with chronic neutrophilic leukemia. 256 Dec 96

A 6-year-old girl with cerebral palsy developed conscious disturbance and generalized convulsion after one-hour hot herb drug bath. Physical examination on admission revealed rectal temperature 41 degrees C, hot skin, respiration 46/min, regular heart beat 98/min, BP 130/60 mmHg, Glascow coma scale 4 (E2M1V1), soft and flat abdomen, no hepatosplenomegaly, no skin rash, no focal neurological sign, increased generalized muscle ton. Laboratory data showed CBC: WBC 20400 cumm (Neutrophils 31%, Lymphocytes 69%), Hb 11.6gm%, ESR 11 mm/hr, arterial blood gas: PH 7.077, PO2 43mmHg, PCO2 57.1mmHg, HCO3- 16 mEq/L, BE-11.5mEq/L, serum sodium 143 mEq./L, potassium 5.2 mEq/L, chloride 101 mEq/L, free calcium ion 3.8mg%, GOT 63IU/L, GPT 263 IU/L, amylase 193 IU/L, alkaline phosphatase 388 IU/L, LDH 1245 IU/L, CPK 677 IU/L, total bilirubin 0.8 mg/dl, direct type 0.1 mg/dl, BUN 18 mg/dl, Glucose 35 mg/dl. Urinalysis revealed proteinuria( ) trace hematuria and pyuria, but no cast. Lumbar puncture is within normal limits. Bacteriology including blood and CSF are normal. Multiple organ failure was noted at that time. Intensive cooling methods were performed including central and peripheral cooling. We used luminal and valium to control the seizure. Condition didn't improve. Afterwards cardiopulmonary arrest developed. Patient expired 8 hours after admission despite of resuscitation. Heat stroke in infancy and childhood is different from that in adulthood. The predisposing factors are high ambient temperature, dehydration, very young baby, sweat gland dysfunction, or ectodermal dysplasia. Definition of heat stroke includes 1) rectal temperature above 41 degrees C, 2) behavioral change, 3) warm skin, wet or dry.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Status epilepticus induced by prolonged immersion in hot herb bath: report of one case]. 263 19

A 67-year-old man was admitted to our hospital with abdominal distension due to hepatosplenomegaly. The peripheral blood revealed Hb content 6.5 g/dl, platelet count 4.7 x 10(4)/microliter, and WBC count 105.8 x 10(3)/microliter with 88% of mature neutrophils. The neutrophil alkaline phosphatase score was 421. Bone marrow aspiration revealed hypercellularity with increased megakaryocytes and myeloid hyperplasia. 46, XY, del 20(q 11) without Philadelphia chromosome was identified by cytogenetic study. The patient was diagnosed as having chronic neutrophilic leukemia and was successfully treated with busulfan, but he died of atypical mycobacteriosis about 20 months later. Analysis of neutrophil function demonstrated diminution of deformability, random mobility, and chemotaxis, but almost normal phagocytosis and bactericidal capacity. Southern analysis showed no rearrangements of breakpoint cluster region (bcr) gene and immunoglobulin heavy chain gene.
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PMID:[Neutrophil dysfunction in chronic neutrophilic leukemia without rearrangements of bcr and immunoglobulin heavy chain genes]. 268 9

A case of primary myelofibrosis complicated with pericardial effusion and proteinuria is described. A 66-year-old female was admitted to our hospital because of abdominal fullness and shortness of breath. On admission, hepatosplenomegaly and pericardial effusion were observed. Blood examination revealed leukoerythroblastic anemia and thrombocytosis with tear drop cells and giant platelets. Bone marrow aspiration was dry tap and its biopsy showed remarkable myelofibrosis. Urinalysis indicated severe proteinuria. Although neutrophilic alkaline phosphatase score was low, no signs of acute blastic crisis of chronic myelogenous leukemia was found. The diagnosis of an atypical type of primary myelofibrosis was obtained. Administration of MCNU was started in August 1987. Hepatosplenomegaly, pericardial effusion and proteinuria were gradually improved after the administration. The etiology of the pericardial effusion and proteinuria were not obvious, however, these facts suggest that these abnormal findings might be related to PMF itself and MCNU was effective to PNF.
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PMID:[The use of MCNU to a patient of primary myelofibrosis complicated with pericardial effusion and proteinuria]. 276 70

A 44-yr-old homosexual male with symptomatic human immune deficiency virus (HIV) infection presented with abdominal pain, hepatosplenomegaly, and rising serum alkaline phosphatase. Laparoscopy revealed multiple purplish nodules on the surface of the liver. Histopathology of the lesions was consistent with Kaposi's sarcoma. Six weeks later, cutaneous Kaposi's sarcoma became apparent on the head and neck. This case is unusual because, in all previously published cases, with the exception of one, intrahepatic Kaposi's sarcoma was found on autopsy. Moreover, all patients had evidence of extrahepatic Kaposi's sarcoma prior to the documentation of liver involvement.
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PMID:Hepatic involvement as the primary manifestation of Kaposi's sarcoma in the acquired immune deficiency syndrome. 281 78

Liver cirrhosis was induced in rats by the combined action of oral phenobarbitone and inhalations of carbon tetrachloride vapors. These rats manifested hepatosplenomegaly, hypoalbuminemia, and 2- to 17-fold elevations in serum transaminases and alkaline phosphatase levels. The hepatic antioxidant enzymes, superoxide dismutase and catalase, showed 28 and 60% decreases, respectively. There was, however, no increase in the hepatic lipid peroxidation. These studies suggest that in cirrhosis liver cell damage may result due to the direct attack of the oxygen free radicals. Lipid peroxidation in the liver may not be a prerequisite for the development of cirrhosis, as is generally believed.
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PMID:Hepatic antioxidant enzymes and lipid peroxidation in carbon tetrachloride-induced liver cirrhosis in rats. 321 29


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