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)

Five cases of histiocytic medullary reticulosis (HMR) are presented and the computed tomographic (CT) findings described. All patients were young adults in their early twenties with fever, malaise and weight loss as their chief complaints. Survival was less than 5 months in all cases. The CT findings were hepatosplenomegaly (5), abdominal lymphadenopathy (5) and bilateral renal enlargement (2). Patchy low attenuation areas in the liver (2), wedge shaped splenic infarcts (2) and focal low attenuation areas in the kidneys (1) were also noted. Ascites (3) and pleural effusion (3) were associated findings.
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PMID:CT features of histiocytic medullary reticulosis. 232 67

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

Transient acantholytic dermatosis is a papulovesicular cutaneous eruption first described in 1970. There have been subsequent reports of similar disorders occurring in patients with malignancy. Angioimmunoblastic lymphadenopathy with dysproteinemia is a disorder characterized by an acute onset of generalized lymphadenopathy associated with fever, malaise, pruritus, night sweats, and hepatosplenomegaly. The patient described had a papular acantholytic dermatosis associated with the development of angioimmunoblastic lymphadenopathy with dysproteinemia-like T-cell lymphoma. The cutaneous manifestations of angioimmunoblastic lymphadenopathy with dysproteinemia are discussed.
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PMID:Transient acantholytic dermatosis associated with lymphomatous angioimmunoblastic lymphadenopathy. 257 18

Clinical, hematologic and hemoglobin composition data on the first case of Hb 0-Arab in association with beta 0-thalassemia in Yugoslavia are reported here. The propositus was a 26-years-old female from Strumica who was admitted to the hospital for several times because of anemia, hepatosplenomegaly, occasional abdominal pains, malaise and fatigue. Laboratory results presented: Hb 10.0 g/dl, RBC 3.84.10(12)/L, PCV 0.260 l/l, MCV 68 fl, MCH 26 pg, reticulocyte count 1.8%, anisopoikilocytosis, polychromasis, numerous target cells, total bilirubin 2.1 mg/dl, (indirect 1.7 mg/dl), serum-Fe 32.3 microM/L. A starch gel electrophoresis of hemolysate provided evidence for the presence of abnormal hemoglobin (approximately 85%) and Hb F (approximately 15%); the Hb A was absent. Familial screening showed her father was heterozygous for the abnormal hemoglobin, whereas the mother was heterozygous for beta-thalassemia. In vitro biosynthesis disclosed a total absence of beta globin and reduced synthesis of beta x x and gamma globin. The alpha/beta x + gamma-globin ratio was 1.77 (normal, 1.0 + 0.1). Amino acid analysis revealed that lysine substituted for glutamic acid at the position one hundred twenty-one of the beta chain (= Hb 0-Arab or beta 121 Glu----Lys).
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PMID:[Hemoglobin O Arab in interaction with beta 0-thalassemia]. 273 98

We reported a rare case of triple cancers with acute lymphoblastic leukemia (ALL) associated with disseminated intravascular coagulopathy (DIC) after the operations of colon cancer and primary lung cancer. A 78-year-old Japanese male, who had been operated upon for colon cancer (adenocarcinoma) on March 1981, metastatic brain tumor (adenocarcinoma) on December 1986, and primary lung cancer (squamous cell carcinoma) on February 1987, was admitted to our hospital because of severe general malaise on December 6 1987. On admission, he had mild hepatosplenomegaly and hemorrhage diathesis such as purpura. Serum LDH increased to 2,515 mU/ml. The white blood cell count was 6,210/microliters with 53% leukemia cells, and the platelet count was 12,000/microliters. A bone marrow was infiltrated with 96.0% leukemia cells. The leukemia cells stained positively for PAS and negatively for peroxidase. Immunological examination of leukemia cells showed that HLA-DR, TdT, B1 and J5 were positive and cytoplasmic Igmu and surface Ig were negative, indicating common ALL. The coagulation studies revealed that the activated partial thromboplastin time was prolonged to 42.0 seconds, FDP increased to 79.9 micrograms/ml, and antithrombin-III decreased to 62%. Chromosome analysis showed a 48, XY, +2, +21q-, t(9;22) karyotype. He was diagnosed as having Ph1 positive ALL associated with DIC. He was treated with vindesine, prednisolone, L-asparaginase, and adriamycin and complete remission (CR) was achieved after two months. But on August 1988, 8 months after CR, ALL and brain tumor relapsed and he died of pneumonia on September 19, 1988.
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PMID:[Ph1 positive acute lymphoblastic leukemia with DIC after operation of colon and lung cancer]. 281 Jul 93

Ten years after the onset of hydroa vacciniforme (HV), a 16-year-old boy developed edema and red induration of the face, ears, and dorsa of the hands. Aggravation of cutaneous manifestations was associated with general malaise, headache, fever, lymphadenopathy, hepatosplenomegaly, and an increase of several serum enzymes. The vesicle was situated intraepidermally with thrombosis and hemorrhage underneath. This confirmed the diagnosis of HV. In addition, dense cell infiltrate was seen in deep dermis and subcutaneous tissue. Histologic and immunohistochemical studies of the dermal cell infiltrate and lymph node showed an infiltrate of helper T lymphocytes with an atypia and histiocytic cells (S100[-], alpha-subunit[+]). Hence, we concluded HV and malignant lymphoma coexisted in this patient.
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PMID:Coexistence of hydroa vacciniforme and malignant lymphoma. 349 Aug 32

An autopsy case of systemic mastocytosis without cutaneous involvement in a 76-year-old woman was described. The patient presented with general malaise, chest and epigastric discomfort, flushing of the face and progressive hepatosplenomegaly, and she terminated in hemorrhagic complications of DIC within 2 months. There was neither rash nor urticaria pigmentosa recognizable in the entire course. The diagnosis was made by the histologic identification of abnormal aggregates of mast cells in a bone marrow aspirate. These mast cell granules were chloroacetate esterase-positive, peroxidase-negative, and electronmicroscopically they were composed of fine granular materials containing variable numbers of lamellar structures. At autopsy, diffuse infiltration of the mast cells was found in the liver, spleen, bone marrow, lymph nodes, lungs, kidneys, stomach, and adrenal glands.
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PMID:Systemic mastocytosis without cutaneous involvement. 355 89

Four weeks after returning from a holiday on Ischia, a 53-year-old man fell ill with nocturnal sweating, fever, severe general malaise and hepatosplenomegaly. Sternal marrow and liver punctures revealed an infection with Leishmania donovani, i.e. Kala-Azar. Characteristic features were hyperplasia of the plasma cells in the bone marrow and liver, together with lymphoplasmocytoid cell wash-out and polyclonal IgG proteinaemia. Treatment with pentamidine (Lomidine) was unsatisfactory, while all manifestations of the disease regressed after two courses of sodium stibogluconate (Pentostam), although the hypergammaglobulinaemia and hyperproteinaemia disappeared only slowly. The plasma-cell hyperplasia in conjunction with the hypergammaglobulinaemia, which suggest multiple myeloma, is interpreted as a result of antibody formation by the chronic parasitic infection. Together with storage-cell hyperplasia it is also taken to be the cause of the peripheral leukopenia and thrombocytopenia.
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PMID:[Kala-azar originating in Ischia]. 365 55

An 8-year-old boy presented with elevated temperature, malaise, hepatosplenomegaly, mesenteric adenitis, and septic shock. Cultures of biopsied abdominal lymph nodes as well as the blood grew Yersinia pestes. The boy's condition improved after two weeks of chloramphenicol and cefotaxime (Claforan). Two days after stopping intravenous antibiotic therapy, the patient again became febrile and complained of abdominal pain. Abdominal imaging with 111In-labeled leukocytes did not show any abnormalities, however, 67Ga-citrate scintigraphy demonstrated an abnormal focus of increased radiopharmaceutical uptake within a confluence of necrosed lymph nodes within the right upper quadrant of the abdomen. In addition, abnormal 67Ga-uptake was seen within the left hip region. Correlative imaging with computed tomography is also presented.
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PMID:Imaging in plague. 366 83

Immunoblastic lymphadenopathy presents with fever, malaise, cough, dyspnea, lymphadenopathy, hepatosplenomegaly, hypergammaglobulinemia, interstitial pulmonary infiltrates, mediastinal adenopathy, and effusions. The diagnosis is made on the basis of lymph node biopsy. Based on the course in our patients and the cases presented in previous reports, a suggested program of treatment for immunoblastic lymphadenopathy includes administration of moderate doses of steroids initially, with a subsequent increase to a higher dosage if desired improvement does not occur. Chemotherapy with three drugs, cyclophosphamide, vincristine, and prednisone, is indicated if remission using steroids fails. The diagnostic dilemma of whether pulmonary infiltrates are due to the disease itself or to pulmonary infection or to cytotoxic changes from chemotherapy always exists and often requires specimens from either transbronchoscopic or open-lung biopsy for definitive diagnosis and treatment.
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PMID:The lung in immunoblastic lymphadenopathy. 727 80


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