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)

A case of malignant histiocytosis in a man of 32 is described. The diseases had a rapid course, showed marked signs of intoxication, generalized lymphadenopathy, hepatomegaly, jaundice, leukopenia, thrombocytopenia. Specific infiltration with atypic histiocytes with phagocytosis of erythrocytes and their debris, leukocytes, and lipids was found in the lymph nodes, skin, bone marrow, liver, kidneys, heart muscle, and along the lung blood vessels. The final diagnosis of malignant histiocytosis is established by histological examination of the affected tissues (most frequently, lymph nodes).
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PMID:[Malignant histiocytosis. Report of a case]. 684 3

A population of patients with agranulocytosis admitted to a general hospital over a period of 12 yr was studied retrospectively in order to determine the causes of the disease. Of the 48 cases identified, 31 (65%) had drug-induced neutropenia, whereas 17 (35%) had chronic neutropenia unrelated to the use of drugs. Eight patients had an underlying hematological malignancy. Patients with agranulocytosis not induced by drugs more frequently had hepatomegaly, splenomegaly, enlarged lymph glands, or thrombocytopenia together with severe anemia. In contrast, drug-induced agranulocytosis was more severe, with a higher incidence of positive blood cultures, low cellularity of initial bone marrow aspirates, and a shorter duration of neutropenia. Dipyrone and methimazole were the drugs most commonly associated with agranulocytosis. Dipyrone was probably the causative agent in two of the seven drug-induced fatalities. In view of these findings, and those of several previous reports, it is proposed that the use of dipyrone in Israel be severely restricted or discontinued altogether.
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PMID:Causes of agranulocytosis in a hospital population: identification of dipyrone as an important causative agent. 685 18

Thirteen patients underwent splenectomy for Gaucher's disease. All patients were Jewish; 12, of Ashkenazi descent, had the chronic (type 1) form, and one child, of Sephardic (Persian) origin, probably had the intermediate (type 3) form. Hypersplenism was the indication for surgery in 11 patients, mechanical problems in the remaining two. The weight of the resected spleens ranged from 1.06 to 13 kg. Following surgery, hypersplenism (thrombocytopenia in particular) was improved, and the mechanical disturbances were relieved in all patients. There were no deaths and no morbidity related to the operative procedure. Long-term follow-up demonstrated progressive hepatomegaly without evidence of hepatic dysfunction in any of the patients. Bone marrow involvement manifested by osteoarticular complications appeared in five patients. Splenectomy is, we believe, a safe mode of treatment for type 1 Gaucher's disease.
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PMID:The role of splenectomy in Gaucher's disease. 687 May 24

The prognostic value of nine clinical and haematological features, recorded at diagnosis in chronic myeloid leukaemia (CML), was analysed in two distinct series of patients. One series (116 cases) was collected at a single hospital over a 12-year period. The second series (139 cases) was collected from a multicentre trial over a 20-month period. Six features were associated with a poor prognosis: splenomegaly (more than 15 cm below the costal margin), hepatomegaly (more than 6 cm below the costal margin), thrombocytopenia (< 150 X 10(9)/l) or thrombocytosis (> 500 X 10(9)/l), a leucocyte count above 100 X 10(9)/l, peripheral blood non-granulated precursors (blast cells) above 1%, and peripheral blood granulated precursors (promyelocytes and myelocytes) above 20%. Depending on the number of negative prognostic factors, patients were divided into three categories: group I (0 or 1 factor), group II (2 or 3 factors) and group III (4,5 or 6 factors). Survival was significantly different in the three groups (P < 0.0005), and this was independent of age (below and above 50). The prognostic value of the classification was confirmed in a third series of 153 patients. We suggest that this classification provides a useful tool to identify prognostic categories in CML, and thus allows a proper allocation of patients to different therapies.
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PMID:Staging of chronic myeloid leukaemia. 693 7

A retrospective was designed to analyse the mode of presentation, clinical signs and haematological and biochemical abnormalities in 225 consecutive Black (Zulu) patients who were admitted to a general medical ward between the years 1970 and 1981 and in whom cirrhosis was later diagnosed. The most common presenting complaint was swelling of the body (60% of the patients), followed by abdominal pain (32%) and episodes of bleeding, mainly from the gastrointestinal tract (19%). On examination, hepatomegaly was encountered in 66% of the patients, with moderate to massive enlargement in 40%. Ascites was detected in 56%, with tense abdominal distension in 34%. Jaundice was present in 38% and emaciation, mental disturbance and splenomegaly in over 25%. Spider naevi (found in 2 patients) and Dupuytren's contracture (found in 1) were very rare. Thrombocytopenia and a high ESR were common. Over 90% of patients had low albumin and high globulin concentrations (albumin less than 20 g/dl and globulin greater than 60 g/dl in 25%). Bilirubin and alkaline phosphatase levels and the prothrombin index were found to be within normal limits in 32%, 24% and 52% of cases respectively. Histologically the lesion was most commonly micronodular (73%) with variable deposits of fat and iron. Peritoneoscopy was the most useful special investigation in the diagnosis of cirrhosis, leading to a correct diagnosis in 77% of cases. In conclusion, the clinical signs, biochemical abnormalities and histological features suggest that the factors causing cirrhosis in the community studied are mixed; it may result from the combined effects of alcohol abuse, malnutrition and chronic viral (e.g. hepatitis B) infections.
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PMID:Clinical presentation and biochemical abnormalities in black (Zulu) patients with cirrhosis in Durban. 707 88

Thirty-six patients with hairy-cell leukemia (HCL) were evaluated, and were divided in two major subtypes: leukopenic (WBC less than 3000/microliters) and non-leukopenic (WBC greater than or equal to 3000/microliters). There were 22 leukopenic and 14 non-leukopenic patients. The leukopenic group were older than the non-leukopenic group, with an average age of 58.4 years compared with 47.6 years. The male/female ratio was higher in the leukopenic (6.3) than the non-leukopenic (2.0) patients. Splenomegaly, hepatomegaly and lymphadenopathy were found in 66%, 32%, and 18% of the leukopenic patients, compared with 92%, 57%, and 35% in the non-leukopenic patients. The leukopenic HCL was associated with more severe anemia, granulocytopenia, monocytopenia, and thrombocytopenia, and higher incidence of serious infections than the non-leukopenic HCL. Increased bone marrow reticulin fibers and unsuccessful marrow aspirations (dry taps) were more frequently associated with the leukopenic than the non-leukopenic HCL.
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PMID:Clinicopathological subtypes in hairy-cell leukemia. 710 11

The natural history of 45 cases of smouldering leukaemia has been studied. Males and females were equally represented, with a median age of 60.5. The median survival of the whole group was only 20 months, but rare cases lived 10 years or longer. 38% developed acute leukaemia; the remainder usually died of the results of marrow failure. Although it was possible to divide these marrow dysplasias morphologically into 3 major subgroups (refractory anaemia with excess of myeloblasts, chronic myelomonocytic leukaemia and chronic erythraemic myelosis), several displayed transitional features. Many showed refractory macrocytosis at diagnosis. The survival of the 3 groups was similar, though patients with high monocyte counts tended to present with less anaemia and fared rather better than the others. Statistical analysis suggests that increasing age, severe anaemia, thrombocytopenia and hepatomegaly are associated with a poor prognosis. Chemotherapy, when attempted, was usually unsuccessful.
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PMID:Natural history of smouldering leukaemia. 715 Apr 70

21 patients (10 male, 11 female) aged between 11 months and 29 years with Shwachman's syndrome are reviewed. All patients had exocrine pancreatic insufficiency. Haematological features included neutropenia in 19 (95%), anaemia in 10 (50%), and thrombocytopenia in 14 (70%); one patient developed erythroleukaemia. Severe infections occurred in 17 (85%) from which 3 (15%) died. Only one child exceeded the 3rd centile for height, and growth retardation was particularly evident in the older patients. All had skeletal abnormalities or delayed skeletal maturation, or both. Metaphyseal dyschondroplasia affected 13 of the older patients and was associated with skeletal deformities. Eight of 9 children under 2 1/2 years had rib abnormalities. Respiratory function tests in children under 2 years demonstrated reduced thoracic gas volume and chest wall compliance. Older patients had reduced forced expiratory volume and forced vital capacity. Neurological assessment showed developmental retardation or reduced IQ assessments, or both, in 85% of patients studied. Other neurological abnormalities included hypotonia, deafness, and retinitis pigmentosa. Neonatal problems had been present in 16 (80%) of the patients and 5 were of low birthweights. Hepatomegaly with biochemical evidence of liver involvement occurred in the younger patients and resolved with age. Other associated features included dental abnormalities, renal dysfunction, an icthyotic maculopapular rash in 13 (65%), delayed puberty, diabetes mellitus, and various dysmorphic features. These findings stress the diverse manifestations of the syndrome and extend knowledge on a number of aspects. Sibship segregation ratios support an autosomal mode of inheritance and an hypothesis for the pathophysiological basis of this syndrome is advanced.
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PMID:Shwachman's syndrome. A review of 21 cases. 743 69

Myelofibrosis with myeloid metaplasia developed during the course of polycythemia vera in a middle-aged man. Severe megakaryocytopenia and thrombocytopenia were early features of this illness and were unresponsive to splenectomy. After splenectomy, hepatic enlargement and acute hepatic failure developed. Extensive extramedullary hematopoiesis dilating hepatic sinusoids and compressing liver cells was the major pathologic finding in the liver. The mechanism of hepatic failure in this disorder is uncertain.
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PMID:Polycythemia vera with myelofibrosis and myeloid metaplasia. Acute hepatic failure following splenectomy. 744 77

A 15-month-old boy with severe aplastic anemia developed veno-occlusive disease (VOD) after allogeneic bone marrow transplantation (BMT), in which the preparative regimen included 50 mg/kg/day cyclophosphamide and anti-lymphocyte globulin for 4 consecutive days. The diagnosis was made based on clinical symptoms and data including, hepatomegaly, right upper quadrant abdominal pain, jaundice, ascites, coagulopathy and thrombocytopenia which was refractory to transfusions of platelet concentrate. We gave 2, 3, 5 and 5 mg/day/body of recombinant tissue plasminogen activator (tPA) followed by heparin and prostaglandin E1 (PGE1) effectively and without significant side effect on days 9, 10, 13 and 14, respectively. Clinical and biochemical improvement was steady and dramatic. We suggest that tPA following continuous heparin and PGE1 infusion may be useful in the treatment of VOD even in infantile cases.
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PMID:Successful treatment of an infant with veno-occlusive disease developed after allogeneic bone marrow transplantation by tissue plasminogen activator, heparin and prostaglandin E1. 763 94


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