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)

Four patients, aged 15-50, with acute lymphoblastic leukaemia (ALL) shown to be of the B-cell type, since they formed rosettes with complement-coated sheep erythrocytes (EAC) and had lymphocytes bearing IgA on the cell surface. Clinically, they had massive leukaemic infiltration associated with hepatosplenomegaly and were extremely resistant to multiple, conventional chemotherapy, as demonstrated by prolonged therapy to achieve a remission or a short-term remission. The surface characteristics of the lymphoblasts in the circulating blood seemed to remain unchanged throughout the course of the leukaemia, despite intensive chemotherapy. The evaluation of surface markers on leukaemic cells might give better information for a forecast of the prognosis of the disease.
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PMID:Acute lymphoblastic leukaemia of the B-cell type refractory to intensive chemotherapy. 30 4

Eighty-nine cases variously diagnosed as histiocytosis X, eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, or malignant histiocytosis from one institution were reviewed. Follow-up data were obtained for all patients. On the basis of clinicopathologic correlation, the following distinct groups were identified. 1. Disseminated histiocytosis (12 patients) with involvement of more than two organ systems at the time of recognition of the disease, affecting children less than 3 years of age (the patient's general condition is poor and hepatosplenomegaly is common. The patients do poorly, and all the patients in this group of the present study died. 2. Histiocytosis confined to bone, whether monostotic (36 patients) or polyostotic (eight patients). This type is self-healing, does not disseminate, and does not require intensive therapy. Clinically the patients are older and in good general condition during the course of the disease. Histologically histiocytes in these two groups are cytologically benign, and the whole process is inflammatory rather than neoplastic. Presence of giant cells, eosinophils, and necrosis are usually associated with better prognosis. Many neoplastic diseases and infectious processes manifest as histiocytosis. More than a third of the cases of this study diagnosed as histiocytosis proved to be other processes. This confusion contributes to differences of various reports and difficulty of interpretation of findings.
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PMID:Idiopathic differentiated histiocytosis. 30 79

This work is a retrospective study of 50 cases of DHX, collected over a period of 27 years. 24 children died, 26 are still alive. The prognosis for DHX was neither dependent on age (usually occurring in children under 2 years) nor on histological findings but on the extent of the lesions. It was possible to establish a clinical staging system distinguishing 2 groups. One, where the disease was severe and almost always fatal, often included the combined symptoms of thrombocytopenia, spontaneous anemia, jaundice, hepatosplenomegaly, respiratory insufficiency and absence of osteolytic lesions. The other, with a favorable prognosis, was characterized by skin lesions, diabetes insipidus, exclusively radiological pulmonary involvement and multiple bone lesions. In cases where death did not occur, DHX was often chronic, frequently persisting for 2 years or more and leading to serious sequelae such as diabetes insipidus, growth stunting, intellectual retardation, blindness or deafness.
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PMID:Disseminated histiocytosis X: analysis of prognostic factors based on a retrospective study of 50 cases. 31 67

Acute myelofibrosis is a rare but distinct accelerated variant of agnogenic myeloid metaplasia that is characterized by marked anemia, peripheral blood myeloblastosis and normoblastosis, a lack of teardrop poikilocytosis, and prominent myelofibrosis. There is usually no palpable hepatosplenomegaly or lymph node enlargement. The clinical course is remarkable short. We describe a 63-year-old man who presented with idiopathic acquired sideroblastic anemia and subsequently developed acute myelofibrosis. Intensive polychemotherapy with vincristine, cytosine arabinoside, and prednisone and a later trial of oxymetholone therapy were ineffective. He died 134 days after the diagnosis of acute myelofibrosis was established. The 11 previously reported cases of acute myelofibrosis are reviewed, and the relationships of acute myelofibrosis to other myeloproliferative disorders and to idiopathic acquired sideroblastic anemia are discussed.
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PMID:Idiopathic acquired sideroblastic anemia terminating in acute myelofibrosis: case report and review of leterature. 31 17

A 38-day-old infant had fever, jaundice, hepatosplenomegaly, and a hemolytic anemia. A peripheral blood smear demonstrated intraerythrocytic malarial parasites identified as Plasmodium vivax. Maternal and infant sera contained antibodies to this species. A directed history revealed the mother had suffered several febrile illnesses in Mexico during her pregnancy. Malaria had not been diagnosed nor was it considered at the time of her delivery at this hospital. Review of this and six other cases of congenital malaria reported in this country since 1950 indicates clinical manifestations seldom appear before 3 weeks of age. Although these signs are more frequently associated with other transplacental infections, their occurrence in an infant whose mother is from or who has traveled in an endemic area should prompt consideration of the diagnosis of congenital malaria.
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PMID:Congenital malaria: a rare cause of splenomegaly and anemia in an American infant. 32 10

Large MtTW15 pituitary tumors produced 200- to 800-fold elevations in serum growth hormone (GH) and prolactin (PRL) levels. Female tumor hosts showed doubling in body weight, milk secretion, and a 2-fold hepatosplenomegaly. Pituitaries of host animals were reduced by about 50% in both weight and concentrations of GH and PRL. Large tumors were well-encapsulated, multinodular and showed variable amounts of necrosis and hemorrhage. Cytofluorometric analysis revealed a range of 100-fold in nuclear DNA content of tumor parenchymal cells which were chromophobic, pleomorphic and frequently mitotic. Concentrations of hormones in tumors were less than in normal pituitaries and highly variable with the ratio of GH/PRL ranging up to 30-fold within the same tumor. Immunostaining and linear scanning quantitation showed that about 50% of the tumor cells contained immunodetectable hormones. Comparison of immunostained adjacent sections showed that hormone-containing tumor cells were pleomorphic, unequally distributed within nodules, lacking in distinctive identifying morphological characteristics and that they contained GH or PRL but not both hormones simultaneously. Collectively our results show that large MtTW15 tumors are comprised of a markedly heterogeneous population of tumor cells and they suggest that the hormone-containing cells are monohormonal secreting tumor cells which can produce GH or PRL but not both hormones.
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PMID:Heterogeneity of the MtTW15 mammosomatotropic tumor. I. Light microscopic evaluation of cell types by means of immunocytochemistry, morphometric quantitation, fluorescence cytophotometry and radioimmunoassay. 34 70

Eight cases of angio-immunoblastic lymphadenopathy were recently diagnosed at the University Hospital of Geneva. The disease was characterized by generalized adenopathy, hepatosplenomegaly, fever, and sometimes skin rash. Six patients had polyclonal dysproteinemia and two displayed autoimmune disorders, e.g. Coombs' positive hemolytic anemia. Lymph node biopsy was a decisive pointer for the diagnosis. Four patients died, two of them from immunoblastic lymphoma. Four other patients are in remission, the longest follow-up being 91 months. Analysis of 213 cases from the literature shows the following features:--Males and females are equally affected, with a predominance after the age of 50.--The onset is characterized by constitutional symptoms (75%) and fever (65%), sometimes following exposure to drugs.--The signs are generalized adenopathy (91%), splenomegaly (66%), hepatomeagly (65%), and skin rash (46).--Polyclonal hypergammaglobulinemia (76%) is found, together with anemia (78%) which is often autoimmune in origin (58%).--The course is often unfavourable and the mortality is over 55%, largely due to opportunistic infections and/or development of immunoblastic lymphoma.
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PMID:[Angioimmunoblastic lymphadenopathy. 8 cases and review of the literature]. 35 86

Specific IgM and IgG antibody to a polysaccharide present in the epithelial cells of the gut of adult schistosomes was measured in four groups of infected patients: I) patients with documented acute schistosomiasis; II) Americans exposed to schistosomiasis within the preceding 0--4 years; III) chronically and heavily infected patients, mostly from Puerto Rico, without hepatomegaly or hepatosplenomegaly; and IV) heavily infected Brazilian children with hepatic or hepatosplenic schistosomiasis. Specific IgM and IgG titers were both highest in the acute Group I patients and lowest in the chronically infected Groups III and IV. Total IgG and IgM levels were compared to specific antibody titers. Immunoglobulin levels tended to follow specific antibody titers except in the chronically infected Groups III and IV in which total IgG rose to high levels. The decrease in specific antigen titers over the course of time occurred despite continued antigenic stimulation and suggests a modulation of the humoral response. The mechanism remains obscure.
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PMID:Antibody response to a polysaccharide antigen present in the schistosome gut. II. Modulation of antibody response. 36 58

Since 1974 an epidemic of tertian malaria has been spreading around the Adana and Tarsus townships in southern Turkey, with a peak incidence of 115 500 cases in 1977. A further increase is to be expected because the insect vectors have become resistant to insecticides. Since 1975 eleven children and three adults have been treated for P. vivax malaria. They had all stayed in the epidemic area during the transmission season which lasts from July to October. Because of a long primary latent period seven patients only developed first manifestations of the disease six to nine months after leaving Turkey. The classical malarial paroxysms were missing during the first weeks of the primary attack. Several children had a febrile illness over weeks with headache, vomiting, abdominal pain, hepatosplenomegaly, high blood-sedimentation rate and severe haemolytic anaemia, so that appendicitis or septicaemia had been suspected. Tetracyclines and trimethroprimsulphamethoxazole were able to suppress the disease without preventing relapses.
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PMID:[Tertian malaria in children and adults from an epidemic region in southern Turkey (author's transl)]. 36 41

A case of congenital malaria due to Plasmodium malariae in one of two living Greek twins born in Germany is presented. In the child fever, anaemia and hepatosplenomegaly were first noticed at the age of two months. Plasmodium malariae parasites were demonstrated in the peripheral blood and bone marrow. The sera of mother and infant contained malaria antibodies. However the mother never showed clinical signs of malaria. Diagnosis and therapy of congenital malaria and the possible mode of transmission of this rare disease are discussed.
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PMID:[Congenital malaria in a Greek twin (author's transl)]. 36 8


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