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)

Eleven cases of myeloproliferative disease occurred in a group of 24 beagle dogs placed in a 60Co gamma-ray field at about 13 months of age and irradiated at an exposure rate of 5 R/22-hour day for duratior of life. Of these 11 dogs, 5 (described in this paper) were diagnosed as having erythroleukemia. The bone marrow showed marked erythroblastic hyperplasia, with maturation arrest of the erythroid elements, and increased numbers of myeloblasts and promyelocytes. The terminal peripheral blood was characterized by marked anemia and thrombocytopenia, with circulating erythrocytic precursors and abnormal erythrocyte morphology. Splenomegaly and hepatomegaly occurred in 4 of the 5 animals. In the spleens and livers of all 5, there was extensive leukemic infiltration and proliferation. The extent of leukemic involvement in other tissues and organs varied in individual dogs.
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PMID:Radiation-induced erythroleukemia in the beagle dog. A hematologic summary of five cases. 26 46

3 patients with acute nonlymphocytic leukemia (ANLL) and an isochromosome (17q) as the sole chromosomal defect are reported. Besides this cytogenetic pattern, they shared several clinical and hematological features such as male sex, advanced age, spleno- and/or hepatomegaly and a suspected preceding myeloproliferative syndrome. Bone marrow cytology was characterized by hypercellularity, prominent baso- and eosinophilia, decreased erythropoiesis and marked increase of dysmorphic megakaryocytes. We suggest that some or most patients with ANLL and i(17q) as the sole cytogenetic defect represent blastic transformation of an underlying chronic myeloproliferative disorder rather than de novo ANLL.
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PMID:Acute nonlymphocytic leukemia (ANLL) with isochromosome i(17q) as the sole chromosomal anomaly: a distinct entity? 236 43

Splenic erythropoiesis was demonstrated by surface counting of 59Fe in 129 of 1,350 ferrokinetic studies performed over a 15 year period. These 129 studies were carried out in 108 patients, including 40 with chronic myelogenous leukemia (CML), 24 with agnogenic myeloid metaplasia (AMM), 18 with polycythemia vera (PV), six with a myelodysplastic syndrome, five with acute leukemia, three with prostate or breast carcinoma, two each with aplastic anemia or Hodgkin's disease, and one each with idiopathic thrombocythemia, multiple myeloma, chronic renal failure, or treated hypopituitarism. Splenomegaly was present in 83% of the studies and hepatomegaly in 72%. Grade II-III myelofibrosis was demonstrated in 62% of the cases. Hepatic erythropoiesis was present in 77% of the studies (only 38% in PV), and marrow erythropoiesis was undetectable in 33%. Total erythropoiesis was about twice normal (range 0.2 to 8 times normal) but was ineffective to varying degrees in 86% of the studies. Relationships between organomegaly, myelofibrosis, and extramedullary erythropoiesis, as well as differences among clinical disorders, are discussed. Differences observed between CML in chronic or blastic phase suggested that the erythroid cell line was involved in the proliferative process. It is concluded that splenic erythropoiesis 1) is encountered in a variety of clinical conditions; 2) is not necessarily associated with splenomegaly or myelofibrosis, even in the myeloproliferative disorders; 3) is part of a predominantly extramedullary (in the liver as well as in the spleen), expanded, and largely inefficient total erythropoiesis; and 4) can be evaluated in a semiquantitative manner by surface counting.
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PMID:Ferrokinetic study of splenic erythropoiesis: relationships among clinical diagnosis, myelofibrosis, splenomegaly, and extramedullary erythropoiesis. 275 9

Although membranous obstruction of the inferior vena cava is frequently assumed to be a congenital defect, it has recently been hypothesized that it could be acquired and secondary to thrombosis at the ostium of hepatic veins. We report the case of a membranous obstruction of the inferior vena cava responsible for isolated hepatomegaly in a young Frenchman. No known cause of thrombosis was found but spontaneous formation of erythroid colonies occurred on bone marrow cultures. This indicated the existence of a latent myeloproliferative disorder, a frequent cause of hepatic vein thrombosis. We suggest that this association of a membranous obstruction of the inferior vena cava with a known, albeit often overlooked, cause of hepatic vein thrombosis provides strong support to the thrombosis theory of membrane formation.
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PMID:Membranous obstruction of the inferior vena cava associated with a myeloproliferative disorder: a clue to membrane formation? 277 25

This report deals with myeloproliferative disorders associated with chronic, persistent Epstein-Barr virus (EBV) infection and with monosomy 7 and aberrations concerning chromosomes 3 and 5. Altogether five children were affected, their age ranging from 1 to 4 years at time of clinical diagnosis. Principal symptoms were: hepatomegaly, splenomegaly, recurring upper respiratory tract infection and anaemia. The serum IgG level remained persistently increased. Anti EBV antibody concentrations were measured over a period of 9 months to 6 years, demonstrating persistently increased concentrations of IgG antibodies to viral capsid antigen (VCA) and against early antigen (EA). In three patients IgA antibodies were also studied and were found to be elevated. Within 2-5 years two children developed chronic myelomonocytic leukaemia from the chronic myeloproliferative syndrome. A third patient who initially was diagnosed as chronic myelomonocytic leukaemia developed acute leukaemia within a period of 12 months. A fourth patient with myeloproliferative syndrome went into spontaneous remission after an observation period of 2 years. A fifth patient, the only one with translocation t(3;5)(q27;q33), displayed symptoms and a clinical course that can best be characterized as juvenile chronic myelocytic leukaemia. The clinical, haematological, serological and cytogenetic findings may be related.
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PMID:Persistent Epstein-Barr virus infection associated with monosomy 7 or chromosome 3 abnormality in childhood myeloproliferative disorders. 298 97

Although cases of hepatic vein thrombosis (Budd Chiari Syndrome) in oral contraceptive (OC) users have been reported in the literature, the association has not been definitively established. Hepatic vein thrombosis, an uncommon disorder, presents with right upper quadrant abdominal pain, hepatomegaly, and ascites. Diagnostic procedures include hepatic scintiscans, ultrasonography, computerized tomography and magnetic resonance imaging, determination of intrahepatic pressure, liver biopsy, and inferior vena cava and hepatic venography. Hepatic vein thrombosis may develop without an apparent underlying cause or as a complication of an illness known to be associated with vascular thromboses such as polycythemia rubra vera or paroxysmal nocturnal hemoglobinuria. In relation to the large numbers of women taking OCs, there have been very few cases of hepatic vein thrombosis. Evidence linking OC use to the development of hepatic adenomas is far more convincing. In a multicenter case-control study of 33 cases of hepatic vein thrombosis in women 15-45 years of age, each of whom was matched to 3-4 controls, the relative risk of hepatic vein thrombosis in OC users compared with nonusers was 2.37 (p 0.02). It was noted that the 1 patient with paroxysmal nocturnal hemoglobinuria, 5 of 12 patients with overt primary myeloproliferative disorder, and 7 of 8 patients with a forme fruste of a myeloproliferative disorder were OC users, suggesting that OCs--through their thrombogenic action--augmented the thrombotic tendency of the underlying condition. The objectives of therapy in hepatic vein thrombosis are to relieve the hepatic congestion and prevent further clot formation. The majority of patients die within 3 years of diagnosis.
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PMID:Hepatic vein thrombosis (Budd Chiari syndrome): possible association with the use of oral contraceptives. 329 14

Sixty-one patients with essential thrombocythemia (ET) were followed from 1974 through 1987 at the Medizinische Poliklinik. Fifty-one patients (84%) presented with thromboembolic complications, and eight patients (13%) with hemorrhages. In seven patients (12%), a thrombocytosis was detected accidentally. Disturbances of the microcirculation (67%), mainly of the fingers and toes (53%), were the most frequent thromboembolic symptoms. The mean age of all patients was 58 years (male patients, 61 years; female patients, 56 years). The average platelet count at diagnosis was 897,000/microliter. The average maximal platelet count was 1.231 X 10(6)/microliter (range, 500,000/microliter to 4 X 10(6)/microliter). Seventy-two percent had a moderate leukocytosis (average, 12,400/microliter), 34% a splenomegaly, 29% a hepatomegaly. Signs of hypermetabolism were infrequent, lactate dehydrogenase (LDH) and uric acid elevations, if present, were moderate. Bleeding time and viscosity were normal in most patients. Spontaneous platelet aggregation was increased in 81% of patients (n = 40). Platelet aggregation studies with the aggregation inducing substances adenosine diphosphonate (ADP), platelet activating factor (PAF), thrombin, collagen, and adrenalin showed hypoaggregation in most patients. Adrenalin-induced aggregation distinguished best between ET-patients and reactive thrombocytosis showing hypoaggregation in all ET-patients tested (n = 16) and in none of 22 controls. Bone marrow studies were performed in 57 patients. The histologic studies (done in 49 patients) were consistent with a chronic myeloproliferative disorder in all cases. In 41 cases (84%) the picture of a megakaryocytic myelosis was found, in 12 of these a granulocyte-rich form of megakaryocytic myelosis. Cytologic studies only (eight patients) did not differentiate ET well from reactive thrombocytosis. Platelet aggregation studies and bone marrow histology may be of help in the diagnosis of difficult cases of thrombocytosis. The Philadelphia status was negative in all cases studied (14 patients). Fourteen patients died. The causes of death were thromboembolic complications in probably 11 and acute leukemia in two patients. The probability of 10-year survival is 64% after a mean follow-up time of approximately 5 years. It appears that considering the average age of ET patients at diagnosis, life expectancy is close to normal.
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PMID:Essential thrombocythemia. Clinical characteristics and course of 61 cases. 336 70

A clinical and hematopathologic review of 66 patients with systemic mast cell disease (SMCD) was undertaken to investigate the frequency and the clinical significance of associated hematologic disorders. Twenty-two patients were found to have a second hematologic disorder, 19 of which involved the myeloid cells (ten dysmyelopoietic syndromes, five myeloproliferative disorders, three acute nonlymphocytic leukemias, and one chronic neutropenia), and three of which involved the lymphoid cells (three malignant lymphomas). A chromosome analysis of the bone marrow revealed abnormalities characteristic of neoplastic myeloid disorders in four patients. Five-year survival for patients with hematologic disorders was 28% compared with 61% for other SMCD patients (P = 0.004). Patients with hematologic disorders differed significantly from other SMCD patients in that they were about 7 years older (P = 0.039), and they presented more commonly with anemia (P less than 0.001) and constitutional symptoms (P = 0.007). These patients also had less frequent skin symptoms (P = 0.003) and urticaria pigmentosa (P = 0.018). By definition, patients with hematologic disorders had a greater percent of hematopoiesis (P less than 0.001) and decreased fat cells (P = 0.011) on bone marrow biopsies. A multivariate model demonstrated that the following independent variables were associated with the presence of hematologic disorders: low hemoglobin (P = 0.001), the absence of hepatomegaly (P = 0.016), high leukocyte count (P = 0.021), and the presence of pathologic fractures (P = 0.051). The frequent coexistence of SMCD with dysplastic and neoplastic disorders of myeloid cells is consistent with the concept that SMCD itself is a disorder of myeloid cells and that the mast cell may be myeloid in origin.
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PMID:Significance of systemic mast cell disease with associated hematologic disorders. 340 77

The authors report 15 cases of nodular regenerative hyperplasia (NRH) of the liver observed in 10 women and 5 men during a 9 year period. Gastrointestinal bleeding due to ruptured esophageal varices revealed the liver disease in 11 cases. Hepatomegaly and splenomegaly were noted in 9 cases and ascites in 7. Anicteric cholestasis was demonstrated in 10 cases. Another disease, e. g. myelofibrosis and monoclonal gammapathy, was present in 11 patients. In 10 patients, portal diversion was performed; outcome being favorable with a follow-up of one to six years. The analysis of these cases and of the 113 previously published reports calls for the following comments: 1) In most cases, NRH is characterized by small-sized hepatocytic nodules scattered throughout the entire liver with no surrounding fibrosis; however this histological pattern may vary somewhat, with adjacent normal zones being found adjacent to typical cirrhotic fibrosis; although a precise morphometric study was not performed in our patients, obstruction of the tiny branches of intrahepatic portal veins was not observed. 2) Histological diagnosis of NRH is difficult and in most cases requires surgical biopsy specimens and specific coloration of the reticulin network. 3) NRH must be considered as a new cause of intrahepatic (sinusoidal or presinusoidal) portal hypertension and/or of chronic anicteric cholestasis. 4) A number of various conditions may be associated with NRH, the most frequent being Felty's syndrome and myeloproliferative disorders. 5) The pathogenesis of NRH remains unknown. 6) Portal diversion generally has a favorable outcome in this disease.
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PMID:[Nodular regenerative hyperplasia of the liver. Study of 15 cases and review of the literature]. 662 10

A total of 134 cases of erythroleukemia (119 from the literature and 15 of the authors' patients) were reviewed in an attempt to correlate survival with age, sex, hepatomegaly, splenomegaly, lymphadenopathy, infection, or hemorrhagic complications at initial presentation; hemoglobin (Hgb), white blood cell (WBC) count, and platelet count; percent myeloblasts in the marrow at diagnosis; and treatment regimens employed. Statistical methods included single classification analysis of variance, nonparametric analysis of variance (Kruskal-Wallis method), contingency table analysis, and correlation coefficient determination for numerical data. No significant correlation between survival and age, sex, hepatomegaly, lymphadenopathy, infection, or hemorrhagic phenomena was found. Improved survival was noted in patients without splenomegaly, and in daunorubicin-treated cases in contrast to those treated with other chemotherapeutic agents. A positive correlation between survival and initial Hgb and WBC was also noted. Erythroleukemia complicating a chronic myeloproliferative disorder had a worse prognosis than de novo erythroleukemia.
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PMID:Prognostic factors and treatment effects on survival in erythroleukemia: a retrospective study of 134 cases. 694 45


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