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)

By the mid 1990s the number of newly diagnosed children with HIV infections in the US may rival the 6,500 children diagnosed with cancer each year. But recent developments in therapy for the child with AIDS offer some hope. A pediatric trial at the NCI used a continuous infusion of azidothymidine in order to achieve the desired blood and cerebrospinal fluid levels. Objective and subjective evidence of response to therapy was noted in all patients who presented with neurodevelopmental deficit. Increases in appetite and weight gain and reductions in lymphadenopathy and hepatosplenomegaly, and increases in CD4 count similar to those observed in adults were apparent. Promising preliminary results have also been seen in trials of dideoxycytidine, dideoxyinosine, and recombinant CD4.
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PMID:Pediatric AIDS: a perspective for the oncologist. 214 35

An important disease entity distinct from cutaneous T-cell lymphoma (CTCL) in Japan is adult T-cell leukemia/lymphoma (ATL), which usually shows the same phenotype as CTCL, i.e., a helper/inducer T-cell phenotype (CD4+CD8-), and usually involves the skin. Clinically, both CTCL and ATL are heterogeneous in nature. In this study, we demonstrated differences between CTCL and ATL in terms of clinical and immunopathologic cell surface features. In patients with ATL, the predominant clinical findings were peripheral lymph node involvement, skin lesions, hepatosplenomegaly, leukemic manifestations, and an aggressive course. In patients with CTCL, by contrast, only skin lesions predominated at the onset of the disease and a relatively good prognosis was demonstrated. Phenotypic heterogeneity of ATL in the skin, i.e., CD4-CD8-, CD4+CD8-, and CD4-CD8+, was demonstrated. Expression of Leu8, CD7 (Leu9), and CD45RA (2H4) was high in both the skin-infiltrating ATL cells and peripheral blood and lymph node ATL cells compared with that in the skin-infiltrating CTCL cells. Expression of CD25 (IL-2R), CD71 (OKT9), HLA-DR, and HLA-DQ was higher in the skin-infiltrating ATL cells than in CTCL cells. Expression of CD29 (4B4) was high, and that of CD45RA (2H4) was low in both the skin-infiltrating ATL and CTCL cells compared with the peripheral blood and lymph node ATL cells. Expression of CD45RO (UCHL-1) was not significantly high in the skin-infiltrating CTCL cells compared with that in ATL cells. The most significant phenotypic difference between ATL cells and CTCL cells was the expression of Leu8 (lymph node homing receptor), CD7 and CD25 antigens on the cell surface, and the main phenotypic difference between skin-infiltrating ATL and CTCL cells and peripheral blood and lymph node ATL cells was the expression of CD29 and CD45RA. These findings confirm that the difference in antigen expression on the cell surface might reflect the clinical features of ATL and CTCL, and suggest that the predominant phenotype of peripheral blood and lymph node ATL cells is that of naive, relatively immature or activated T-cells, and that CTCL cells are previously activated (memory) T-cells. In other words, CTCL cells do not share the same origin as ATL cells. These observations support the concept that ATL is a disease distinct from CTCL.
Cancer 1990 Dec 01
PMID:Comparative study of cutaneous T-cell lymphoma and adult T-cell leukemia/lymphoma. Clinical, histopathologic, and immunohistochemical analyses. 224 93

Three children with acute lymphoblastic leukemia developed disseminated fungal disease predominantly involving the liver and spleen. The three patients were undergoing induction chemotherapy and had neutropenia when they presented prolonged fever not responsive to antibiotics. Once neutropenia was recovered, hepatosplenomegaly leukocytosis, elevated serum alkaline phosphatase, and hypoechoic areas in the spleen and liver ultrasound were observed. All fungal blood cultures were negative, with the diagnosis being confirmed by histologic study. One of the patients died without achieving control of the candidiasis. The other two patients received prolonged antifungal treatment concurrently with chemotherapy and both are alive, one of them cured and in complete remission. The increasing frequency of this infection in recent years and the importance of a prompt and prolonged administration of antifungal therapy to obtain the cure are discussed.
Cancer 1990 Feb 15
PMID:Hepatosplenic candidiasis in children with acute leukemia. 229 57

Bone marrow monosomy 7 is the most frequent karyotypic abnormality found in patients with chronic myeloproliferative disorders. To a review of 46 previously reported pediatric patients we add three additional cases. Clinical presentation is usually dependent upon which cell lines are most perturbed in this pluripotent stem cell disorder. Sixteen (35%) children presented by their first birthday and 35 (76%) by their sixth birthday. Distinctive differences in presentation exist between infants, children, and adolescents. Younger patients were more symptomatic and had greater degree of hepatosplenomegaly and leukocytosis. The prognosis is very poor and death usually occurs within two years from complications attributable to cytopenias, cellular dysfunction, or transformation to acute nonlymphocytic leukemia. Implications for therapy are discussed.
Cancer Genet Cytogenet 1990 Feb
PMID:Monosomy 7 syndrome. Clinical heterogeneity in children and adolescents. 229 85

Systemic candidiasis with Candida-induced abscesses, predominantly in the liver and the spleen, was diagnosed in 27 patients with haematologic malignancies after intensive cytostatic therapy. Specific features included septic fever unresponsive to antimicrobial therapy, hepatosplenomegaly with multiple lesions in the liver and spleen (diameter up to 2 cm) as detected by computed tomography (CT) or ultrasound, and an elevation in liver enzymes. During treatment, induced neutropenia, hepatic and splenic foci were poorly defined histologically and were not identified by imaging procedures. After granulocyte recovery these foci showed characteristic histological patterns. Ultrasound and/or CT investigations of the abdomen now revealed characteristic lesions in the liver and the spleen. Gamma-GT and alkaline phosphatase were early indicators of hepatic involvement in Candida septicaemia and were often already elevated in aplasia.
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PMID:Hepatosplenic candidiasis, a late manifestation of Candida septicaemia in neutropenic patients with haematologic malignancies. 233 85

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

A 7 week old baby presented with generalized lymphadenopathy, obstructive jaundice and massive hepatosplenomegaly. The clinical picture closely resembled a case of disseminated malignancy. Soon after the diagnosis of tuberculosis was made by pathological examination of a lymph node, the patient died. An autopsy revealed primary tuberculous foci in the liver and massive lymphadenopathy at the porta hepatis consistent with transplacentally acquired tuberculosis. The importance of early diagnosis and treatment of this rare condition is emphasized.
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PMID:Congenital tuberculosis. 261 41

A 16-month-old girl presented with hepatosplenomegaly and pancytopenia. Bone marrow aspiration showed a florid increase in macrophages with marked hemophagocytosis. She subsequently improved spontaneously with no therapeutic intervention, but 2 months later presented with frank acute monoblastic leukemia. This case illustrates the difficulties in classifying malignancies of the monocyte-macrophage lineage and how hemophagocytosis can be the presenting feature of a range of diseases.
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PMID:Hemophagocytosis and acute monoblastic leukemia. 264 6

A 37-year-old male was admitted to hospital because of a right hypochondrial pain and icterus. His physical examination showed hepatosplenomegaly, and the laboratory findings demonstrated abnormal hepatic and pancreatic functions. A CT examination revealed a large mass of a low density in the pancreas head and tail. Further, an immunological study revealed that the patients serum Ca 19-9 level was elevated, but that the CEA and AFP levels were normal. Both the pathological and cytological examinations, however, did not indicate a malignancy of the pancreas. The patient subsequently developed uremia, a hemorrhagic tendency, and then died. An autopsy confirmed a pancreatic tumor which occupied the head of pancreas. Histologically, the tumor contained round cells with scanty cytoplasms and showed a sarcomatous pattern. An immunohistochemical study showed that the LCA, MB-1, and the LN-1 for B cell markers were positive, while the MT-1 for T cells was negative. The case illustrates a malignant lymphoma of the pancreas which demonstrated a serum Ca 19-9 elevation.
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PMID:[An autopsied case of malignant lymphoma of the pancreas with an elevation of the serum Ca 19-9 level]. 269 18

Circulating immune complexes (CIC) were measured at the time of diagnosis in 81 patients with acute leukemia or blast crisis of chronic myeloid leukemia using precipitation by 3.75% polyethyleneglycol. Elevated CIC levels did not adversely influence complete remission duration and survival, patients with normal CIC levels exhibited mostly shorter remission and survival than those with elevated or borderline levels. No significant correlation was observed between CIC levels and Hb, WBC, CBC, platelet count, age, serum bilirubin, total protein, fibrinogen, AST and ALT levels, presence of hepatosplenomegaly and/or lymphadenopathy, HbSAg positivity, complete remission duration and survival. The lack of correlation may be caused by altered immune response in leukemic patients, but the obtained results may also be affected by the nonspecific nature of the method used for the detection. Simultaneous detection of CIC levels by multiple tests and evaluation not only of the number but also of the composition and size of CIC may decrease the incidence of false results. Nevertheless, only the establishment of antigen-specific assays may resolve the controversies in the detection of CIC and thus contribute to a more precise assessment of the role of CIC in prognosis of cancer, as well as to the verification of reliability of using CIC as a tumor marker.
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PMID:Circulating immune complexes in acute leukemia. 270 22


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