Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

B-cell lymphoma associated with haemophagocytic syndrome (HPS) is extremely rare in Western countries but has recently been increasingly reported in Asian countries. We describe seven patients with B-cell lymphoma associated with HPS, six males and one female, age range 41-82 years (median 63 years). All patients had fever and splenomegaly, and six of the seven patients had hepatomegaly with no associated lymphadenopathy. The bone marrow showed haemophagocytosis and an infiltration of lymphoma cells. All patients showed increased levels of lactate dehydrogenase, C-reactive protein, ferritin and soluble interleukin-2 receptor. Lymphoma cells were positive for CD19. CD20 and surface immunoglobulin in all patients examined, and positive for CD5 in four of seven patients. Cytogenetic analyses of bone marrow cells showed a complex structural abnormality including chromosome 14q32 in two patients, 19q13 in three patients and deletion of the terminal part of 8p21 in six patients. The prognosis was poor; only two of the seven patients have survived in complete remission with a median survival of 11 months. These data suggested that B-cell lymphoma associated with HPS might constitute a distinct biological and clinical disease entity. Abnormality of chromosome 19q13 and loss of 8p21 might be involved in the pathogenesis of this disease.
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PMID:B-cell lymphoma associated with haemophagocytic syndrome: a clinical, immunological and cytogenetic study. 1052 18

A 34-year-old obese woman with human immunodeficiency virus (HIV) infection diagnosed a year earlier was seen because of nausea, vomiting, and intermittent diarrhea for 3 weeks. Her current medications included zidovudine. Physical examination revealed tachypnea and tender hepatomegaly. Computed tomography of the abdomen showed hepatomegaly with fatty infiltration. Liver enzymes were within normal range except for elevated lactate dehydrogenase (LDH). The serum bicarbonate value was low, with a lactate level three times normal. The tachypnea and dyspnea worsened as lactate concentrations rapidly increased to 15 times normal. Although her Po2 and cardiac index were initially adequate, the patient had acute respiratory failure. She died with multiorgan dysfunction, including hepatic failure, severe lactic acidemia, disseminated intravascular coagulation, and renal failure. Autopsy revealed hepatomegaly and massive steatosis. Physicians should consider lactic acidosis in patients taking zidovudine and having unexplained tachypnea, dyspnea, and low serum bicarbonate concentrations.
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PMID:Zidovudine-associated type B lactic acidosis and hepatic steatosis in an HIV-infected patient. 1021 65

The authors present the characteristics of a group of 23 patients with mantle cell lymphoma. In the group only a slight predominance of men over women was found (1.1:1), the median age was 63 years. Twenty-one (91%) of the patients were diagnosed in stage IV (Ann Arbor). In all these patients the bone marrow was affected. In 19 of them immunoflowcytometric analysis revealed the typical clone of B lymphocytes (CD5 positive)/CD 23 negative). The majority of patients had at the time of diagnosis a large tumourous mass with massive splenomegaly (61%), hepatomegaly (57%) and bulky disease (52%). The node was excised in 17 patients, but in four patients (24%) during the first session the diagnosis was not assessed correctly. In the laboratory findings an inclination to anaemia, thrombocytopenia, lymphocytosis and in particular to high levels of serological indicators of activity of the disease dominated--lactate dehydrogenase, beta-2-microglobulin and serum thymidine kinase. All patients were treated by chemotherapy. Complete remission was achieved by the date of evaluation in one patient (4%), partial remission in seven patients (30%) but 48% patients did not respond to first line treatment. Nine patients of the group died, their median of survival was 14 months (0-24), the median of the follow up of the remaining patients was 133 months (2-31). Two female patients had large-dose treatment with subsequent administration of autologous stem cells. The first one is after 370 days of treatment in complete remission, the second one developed a relapse 100 days after the procedure. From the results and analysis of the literature ensues that mantle cell lymphoma is one of the aggressive malignant B-lymphoproliferations with a very adverse prognosis and it deserves therefore special diagnostic and intense therapeutic attention.
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PMID:[Mantle cell lymphoma as a diagnostic and therapeutic problem]. 1042 28

B-cell lymphoma-associated hemophagocytic syndrome (B-LAHS) is extremely rare in Western countries but has recently been increasingly reported in Asian countries, especially Japan. Here, we reviewed 25 previously reported Japanese cases of B-LAHS and summarized its clinicopathologic features and therapeutic outcome. The median age of onset was 63 years old with initial presentation of fever, hepatomegaly, and splenomegaly without associated lymphadenopathy. Laboratory findings showed increased levels of lactate dehydrogenase, C-reactive protein, ferritin and soluble interleukin-2 receptor. Histopathologically, hemophagocytosis was often seen in the bone marrow and spleen. Various percentages of lymphoma cells were seen in the bone marrow, positive for CD19, CD20 and surface immunoglobulin. and some were also positive for CD5. Cytogenetic analysis showed a complex structural abnormality including chromosome 14q32, 19q13 and deletion of the terminal part of 8p21. Some patients had histological features of intravascular lymphomatosis (IVL). The prognosis was poor with a median survival period of 9 months. We treated five patients using autologous peripheral blood stem cell transplantation (PBSCT), and four are still in complete remission nine to 24 months after PBSCT, suggesting that high-dose chemotherapy followed by PBSCT might improve the survival rate.
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PMID:B-cell lymphoma-associated hemophagocytic syndrome. 1081 54

A rare case of thrombotic microangiopathy in a patient with hemophagocytic syndrome is reported. An 18-year-old girl was admitted following prolonged fever, watery diarrhea, abdominal discomfort, and a 2-week history of rhinorrhea, cough, and painful cervical lymph nodes. Anemia, thrombocytopenia, jaundice, hepatomegaly, and mild azotemia developed within 2 weeks of admission. The diagnosis of a reactive hemophagocytic syndrome, probably secondary to infection, was made based on the findings of bone marrow examination. Extensive investigation failed to identify a causative agent. The disease initially responded rapidly to intravenous steroids and high-dose immunoglobulin therapy but relapsed soon after tapering of the steroids. Although her condition improved again on resumption of treatment with high-dose steroids, nephrotic range proteinuria and microscopic hematuria developed after the steroids were tapered. Fragmented erythrocytes were seen in peripheral blood with elevated serum lactate dehydrogenase and decreased serum haptoglobin concentrations. The results of subsequent renal pathology examination were also compatible with thrombotic microangiopathy. The disease course finally stabilized after a course of pulse methylprednisolone therapy. Immune hyperactivity, particularly hypercytokinemia and monocyte hyperactivity, could have accounted for the development of thrombotic microangiopathy in this case. Only strong immunosuppressive therapy can control such disease activity.
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PMID:Thrombotic microangiopathy in hemophagocytic syndrome: a case report. 1210 56

Primary plasma cell leukemia (PPCL) is a rare form of disease accounting for 1-2 percent of myelomas. Between September 1990 and November 2000, among 540 patients with myeloma studied, 24 fulfilled the criteria of PPCL (4.4 percent). We found high frequencies of female patients (62 percent), Bence Jones proteinuria (79 percent), anemia (88 percent), bleeding (54 percent), confusional syndrome (42 percent), weight loss (71 percent), hepatomegaly (25 percent), splenomegaly (21 percent), leukocytosis (62 percent), and thrombocytopenia (71 percent). High serum levels of creatinine, calcium, lactate dehydrogenase (LDH), and beta(2)-microglobulin were detected in 50 percent, 37 percent, 58 percent, and 71 percent, respectively. Four patients were treated with vincristine, melphalan, cyclophosphamide, prednisone, and adriamycin (VMCPA), 12 with vincristine, adriamycin, and dexamethasone (VAD), and 8 with M-80 (oral melphalan 80 mg/m(2) plus dexamethasone 40 mg/m(2)). There was a trend toward lower values of Karnofsky score (P=0.07) and higher values of LDH (P=0.2) in the VAD group. Other clinical characteristics were comparable among the three groups. Complete plus partial responses were achieved in one and six patients treated with VMCPA and M-80, respectively. All patients treated with VAD failed to respond to treatment. Patients receiving the M-80 regimen experienced higher platelet toxicity (P=0.05), vomiting (P<0.0003), and mucositis. Also, the need for red blood cell transfusions was higher in the M-80 group. Median overall survival was 60 days. Overall survival was better in patients achieving complete or partial response. In conclusion, our study illustrates that intermediate doses of melphalan plus dexamethasone are an effective chemotherapy regimen for this aggressive disease. Response to treatment is the only prognostic factor for survival in these patients.
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PMID:Intermediate doses of melphalan and dexamethasone are better than vincristine, adriamycin, and dexamethasone (VAD) and polychemotherapy for the treatment of primary plasma cell leukemia. 1218 4

A 24-year-old man was admitted to our hospital because of liver dysfunction. He had been diagnosed as having psoriasis vulgaris at 18 years of age. Physical examination demonstrated obesity, general erythema, and hepatomegaly. Laboratory data revealed elevated serum levels of aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, and glucose. A histological examination of the liver revealed macrovesicular fatty change and infiltration of inflammatory cells, including lymphocytes and polymorphonuclear cells, within the liver lobules. Pericentral fibrosis and pericellular fibrosis were also recognized. He was diagnosed as having nonalcoholic steatohepatitis (NASH), based on the fact that he had no habit of drinking alcohol, as well as psoriasis vulgaris and diabetes mellitus. We herein report a very rare case of NASH associated with psoriasis vulgaris.
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PMID:Nonalcoholic steatohepatitis associated with psoriasis vulgaris. 1558 Apr 5

A 16-year-old Caucasian girl of Albanian origin was admitted to the hospital complaining of intermittent fever (38 degrees C) for a week, nausea, vomiting, and abnormal laboratory findings (elevated serum aminotransferases levels AST/ALT 77/40 U/l and erythrocyte sedimentation rate 80 mm/first hour, as well as leukopenia 2.5 x 10(3)/mm3), which were found in a blood examination. Physical examination revealed slight hepatomegaly and splenomegaly, as well as cervical and axillary lymphadenopathy. A diagnostic open lymph node biopsy was performed and Kikuchi-Fujimoto disease (KFD) was established based on the characteristic histological pattern. Other abnormal laboratory findings were C-reactive protein 6.8 mg/dl and serum lactate dehydrogenase 900 U/l. Her history included a diarrhoea syndrome 2 months before the present admission, during the summer holidays, for which she was treated with metronidazole. At that time, characteristic cysts of giardia lamblia intestinalis were observed in the stools. Herein, we present this case hypothesising that the protozoal infection caused by the giardia lamblia intestinalis was probably triggering an immune response leading to KFD. The patient's age in combination with this firstly reported protozoal pathogen, as a triggering agent leading to KFD, consist a very interesting originality. Additionally, some review data is also given.
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PMID:Giardia lamblia intestinalis: a new pathogen with possible link to Kikuchi-Fujimoto disease. An additional element in the disease jigsaw. 1564 21

Liver involvement in tuberculosis in absence of miliary tuberculosis is rare. This study was performed to analyse the spectrum and response to treatment of hepatic tuberculosis in the absence of miliary abdominal tuberculosis. Retrospective analysis of seven cases of hepatic tuberculosis without miliary abdominal tuberculosis who presented at the single tertiary referral center were analyzed. All patients presented with fever and hepatomegaly. Five of them had pain in upper abdomen and vomiting. HIV serology was positive in one patient. All patients had normocytic normochromic anaemia, raised erythrocyte sedimentation rate (Mean 65). Mild elevation of liver enzymes and low albumin (Mean 2.4 gm%) with reversal of albumin globulin ratio (Mean 0.6) were seen in all. Two had jaundice. Prothrombin time was normal in all and lactate dehydrogenase values were elevated in all (Mean 794 IU/L). On ultrasonography, 2 had multiple hypodense lesion, 1 had coarse echotexture of liver, 1 had hyperechoic pattern and 3 had just hepatomegaly. Complete resolution of liver lesions on treatment with 4-drug anti-tuberculosis drug chemotherapy was seen. In conclusion, liver tuberculosis has protean manifestations with nonspecific alteration of liver function tests and is best diagnosed on liver biopsy. Overall response to therapy is satisfactory.
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PMID:Hepatic tuberculosis in absence of disseminated abdominal tuberculosis. 1653 64

Angioimmunoblastic T-cell lymphoma most often affects the elderly. Patients present with generalized lymphadenopathy and systemic symptoms; half also have hepatomegaly, splenomegaly and a rash. Polyclonal hypergammaglobulinemia, elevated lactate dehydrogenase, and anemia are the main laboratory abnormalities. Autoimmune phenomena (including autoimmune hemolytic anemia, immunologic thrombocytopenia, and autoantibodies) are common. Lymph node biopsy is needed to confirm this diagnosis. Genetic analysis that reveals a monoclonal T-cell population is also relevant. The underlying immune deficiency explains the frequency of infections. Most patients are treated with combination chemotherapy. Autologous stem cell transplantation is proposed to the youngest. Immunosuppressive drugs may be appropriate for elderly or relapsing patients. The overall 5-year survival rate is 30%.
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PMID:[Angioimmunoblastic T-cell lymphoma]. 1758 41


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