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)

Thirty-nine patients with chronic granulocytic leukemia (CGL) in blastic crisis (BC) were studied from 1981 to 1988 at the Hematology Service of the General Hospital of Mexico. The patients were from 18 to 80 years old. Twenty-one patients (54%) were in lymphatic BC and 18 patients (46%) corresponded to BC myeloid. All the patients were treated with different chemotherapy schedules. Only three patients in lymphoid BC and two in myeloid BC achieved complete remission. The longest remission time was 24 weeks and the longest survival 36 weeks. The clinical and laboratory features, such as age, anemia, bleeding, fever, bone pain, adenopathy, splenomegaly, hepatomegaly, extramedullary infiltration, leukocyte count, hemoglobin, platelets, blast cells, in peripheral blood and bone marrow, basophils, and morphology and cytochemistry stains characteristic in bone marrow, were compared between the two groups of patients. None of the clinical and laboratory findings studied were significantly different between the two types of BC, except the evolution time from the diagnosis to the BC, which was more than than two years for most of the patients in lymphoid BC. We also studied the prognosis factors related to survival time. There were no clinical or laboratory differences among the patients who survived more than or less than 14 weeks.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Chronic granulocytic leukemia in blastic crisis. Prognostic factors. 189 82

Forty-eight Japanese infants with acute lymphoid leukemia (ALL) (n = 24) and acute nonlymphoid leukemia (ANLL) (n = 24) were analyzed on the basis of clinical and laboratory data. Morphologically, 20 of the 24 infants with ALL were of the FAB L1 type, and 20 of the 24 infants with ANLL were of the M4 or M5 type. Markedly enlarged liver and spleen, and hyperleukocytosis (more than 50,000/microliters) were seen in 9, 12, and 14 infants with ALL and 10, 11, and 10 infants with ANLL, respectively. Initial CNS leukemia was evident in 2 infants. Chromosome studies of the leukemic cells showed abnormal karyotypes in 9 of the 21 infants with ALL and 19 of the 22 infants with ANLL, consisting mainly of translocation 11, 12, and inversion 16. By surface marker analysis, only 7 of the 22 infants with ALL (32%) were diagnosed as having common ALL (HLA-DR+, CD19+, CD10+). Of the 15 infants with ANLL, 12 and 5 infants also showed reactivity to HLA-DR and CD19, respectively. All of the 5 ANLL infants with lymphoid markers showed different leukemic cell features at the time of relapse. Twelve of the 19 infants with ALL (63%) who achieved a complete remission relapsed within the first 2 years; 8 of the 21 with ANLL (38%) relapsed within the first year. Analysis of event-free survival shows that the ALL infants with hyperleukocytosis have a poorer prognosis than those without hyperleukocytosis (p less than 0.05). Infant leukemia originates in a multipotent cell with lymphoid and myeloid features, and intensive multiagent chemotherapy is necessary for the treatment of infants with acute leukemia.
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PMID:Infant leukemia in Japan: clinical and biological analysis of 48 cases. 199 Feb 55

The T cell chronic leukemias encompass a broad spectrum of diseases involving mature post-thymic T cells. With the development of highly specific marker studies, clear patterns of immunophenotypic and functional characteristics of the involved cells have emerged. These studies, along with the development of molecular probes for the T cell receptor gene loci, have helped to elucidate the pathogenetic basis for the highly variable clinical course which has been described for patients with these disorders. The T gamma lymphocytosis syndrome has been identified as a benign chronic illness which is nevertheless usually a monoclonal neoplastic proliferation of large granular lymphocytes. These patients represent a distinct clinical entity characterized by splenomegaly, neutropenia, and peripheral blood lymphocytosis. The cells of TGLS are large granular lymphocytes and display many of the immunophenotypic and functional characteristics of NK and K cells. These cells have been implicated pathogenetically in the associated cytopenias seen in the illness, but a clear link has not been established. Although the lymphoproliferative manifestations of the disease are usually easily controlled with low-dose alkylating agents, therapy of the neutropenia has been relatively unsuccessful. Separating these patients from the rest of the spectrum of the T cell chronic leukemias has provided insight into the other disorders as well. It has established that T-CLL and T-PLL are, in fact, extremely rare. T-CLL is similar to its B cell counterpart, except that patients have a higher incidence of skin infiltration. Available data suggest that the prognosis in T-CLL is actually less variable, and somewhat worse, than generally believed when those patients were viewed in conjunction with the patients with the more benign TGLS. T-PLL is an extremely aggressive disease characterized by massive splenomegaly, lymphadenopathy, and skin infiltration. It is refractory to most forms of therapy. These illnesses are again phenotypically distinct from the retrovirus-associated ATLL. Most of the early cases of T-CLL reported from Japan were probably ATLL; this disease is characterized by pronounced splenomegaly, hepatomegaly, lymphadenopathy, and skin infiltration. It has an extremely aggressive natural history, and survival is usually less than 1 year from diagnosis. The rapid development of sophisticated immunologic and molecular techniques for analyzing T cell proliferations has allowed highly specific distinctions to be made among the cells of origin of the different T cell chronic leukemias. It is hoped that increased understanding of the immunologic and functional characteristics of these diverse T lymphoid populations will provide further insights which will have an impact on directed therapeutic interventions in the future.
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PMID:T gamma lymphocytosis and T cell chronic leukemias. 218 3

We present two cases of primary malignant lymphoma of the liver, a very unusual localization of primary malignant lymphoma since only 31 similar cases were reported in the literature. The main symptoms included: hepatomegaly, fever, slight cholestasis and non specific alteration of the liver appearance on ultrasonography or computed tomodensitometry examination. Diagnosis was established in both cases by histopathological demonstration of infiltration by large lymphoid cells. Immunophenotyping in one case confirmed a B cell phenotype. Primary malignant lymphomas of the liver are high-grade malignant large-cell tumours and the immunophenotype, when known, is of the B type. The prognosis of these tumours is not well known due to their extreme rarity. They appear to be highly chemosensitive and cure may be obtained in the long-term in some cases. These data justify careful histological assessment in all cases of hepatomegaly of a tumoral nature.
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PMID:Primary malignant lymphoma of the liver. Report of two cases and review of the literature. 219 36

A 48-year-old male was admitted to our hospital on April 20, 1989 because of general fatigue and abdominal fullness. Physical examination showed hepatomegaly, massive splenomegaly, and systemic lymphadenopathy. Hematological findings revealed WBC 73,000/microliters, RBC 289 x 10(4)/microliters, Hb 8.0g/dl, and platelet 9.1 x 10(4)/microliters. WBC differential count demonstrated a mixture of 63% matured small lymphocytes and 32% prolymphocytoid cells. Bone marrow aspiration was unsuccessful with a dry tap. Surface marker analysis of peripheral blood lymphoid cells disclosed that they were positive for anti-HLA-DR, CD 5, CD 19, CD 20, CD 21, CD 25, Sm-IgM, Sm-IgD, and Sm-K. He was diagnosed as B-CLL/PL, and treated with VEPA with partial remission. CLL/PL which was advocated by Melo in 1986 is regarded as a distinct clinical entity intermediate between CLL and PLL in clinical and laboratory features. Our case is interesting with regard to good response to combination chemotherapy, though most cases of CLL/PL have a resistance to standard chemotherapy.
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PMID:[B-chronic lymphocytic leukemia/prolymphocytic leukemia (CLL/PL)--a case report]. 228 70

Weanling male Fischer 344 rats were exposed daily by gastric intubation for up to 15 weeks to the polychlorinated biphenyl (PCB) Aroclor 1254 at 0.1, 1, 10, or 25 mg/kg body weight. At 5, 10 and 15 weeks groups of rats were killed and immune functions were evaluated. The immune parameters examined included the following: body and lymphoid organ weights, mitogen-stimulated lymphoproliferative (LP) responses, natural killer (NK) cell activity, mixed lymphocyte reaction (MLR), and cytotoxic T lymphocyte (CTL) response. After 15 weeks of dosing body weights were reduced in rats receiving 25 mg/kg PCB while thymus weights were decreased in rats receiving 10 and 25 mg/kg. NK cell activity was reduced in rats dosed for 15 weeks at 10 and 25 mg/kg. The LP response to phytohemagglutinin was enhanced in rats dosed for 15 weeks at 25 mg/kg PCB. Exposure of rats to PCB did not affect the MLR or CTL responses. Other groups of rats were exposed to cyclophosphamide (CY) and served as positive controls for the immune assays employed. CY induced alterations in all of the immune parameters measured, indicating that this is an appropriate battery of immune function tests which is capable of detecting immune alterations in the rat. Alterations in immune function induced by daily gastric intubation with PCB were accompanied by reductions in body weight and/or hepatomegaly. These results suggest that the observed immune alterations may be related to the overt toxicity of this PCB in the rat.
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PMID:Evaluation of the immunotoxicity of low level PCB exposure in the rat. 249 55

Three cases of a syndrome featuring massive splenomegaly, gross generalized lymphadenopathy, and moderate hepatomegaly are reported. Spleen weights ranged from 800 to 2400 g. Gradual depletion of lymphoid germinal centers, and prominent infiltration of the splenic and lymph node cords with plasma cells, immunoblasts and actively dividing B cells were the most distinctive histological features. The liver in two cases showed portal infiltrates. A marked hypergammaglobulinemia, a decrease in blood cholesterol level and hematological abnormalities related to hypersplenism were observed. The condition begins early in life and runs a chronic course, of up to 25 years. There was a family history in only one instance. Since there was no generalized immunodeficiency nor local depletion of T cells or dendritic reticulum cells, a failure in the local regulation of the immune response and possible cytokine production is postulated. This condition underlines the pivotal role of the local organization of the germinal centers in cellular cooperation and in the carrying out and regulation of the immune response.
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PMID:The disappearance of germinal centers in chronic lymphadeno-hepato-splenomegaly syndrome in childhood: report of three cases. 271 99

Adult wild-trapped opossums were infected with Leishmania donovani (Khartoum strain, WR 378) and evaluated as an animal model of visceral leishmaniasis. All infected opossums died within 32 days. Loss of body fat, hepatomegaly, and petechiae of skin and abdominal musculature were seen at necropsy. Microscopically, numerous amastigote-laden macrophages were seen in histologic sections of liver, spleen, and lymph nodes; fewer parasite-laden macrophages were in the bronchial-associated lymphoid tissues and renal glomeruli. Hematological findings included thrombocytopenia (terminal), neutropenia, and lymphopenia. Blood lymphocyte blastogenesis in response to concanavalin A and phytohemagglutinin was decreased markedly at day 24 post-infection (PI). Serum antibodies (1:40 dilution) to promastigotes of L. donovani were detected in five of eight infected opossums tested on days 10 and 24 PI. Total bilirubin concentrations and alanine aminotransferase and aspartate aminotransferase activities were increased after day 25 PI. Activated partial thromboplastin times and one-stage prothrombin times were prolonged before death. Concurrently, factors V, VIII, and XII activities were decreased.
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PMID:Experimental visceral leishmaniasis in the opossum. 276 21

Sixty cats with hematologic abnormalities indicative of non-lymphoid hematopoietic neoplasia were classified into two groups, myelodysplastic syndromes (MDS) and acute myelogenous leukemias (AML), using criteria developed for human patients with similar diseases. Cats with myeloblast counts in bone marrow of less than 30% were classed as MDS and cats with myeloblast counts of 30% or greater were classed as AML. The clinical, laboratory, and postmortem findings in each group were described and compared. Clinical signs of disease were similar in both groups, the most common being inappetance, lethargy, and weakness. Non-regenerative anemia, macrocytosis, neutropenia, and thrombocytopenia were frequent hemogram abnormalities in both groups. Diagnostically useful differences in physical and peripheral blood findings were a higher prevalence of splenomegaly and/or hepatomegaly, thrombocytopenia, and severe anemia in the AML group. Circulating myeloblasts were found only in cats in the AML group. Outcome of disease was similar in both groups; 85% of the cats in each group died or were euthanatized within one week of diagnosis. In cats that were necropsied, extramedullary leukemic infiltrates were found in all cats in the AML group and in none of the cats in the MDS group.
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PMID:Non-lymphoid hematopoietic neoplasia in cats: a retrospective study of 60 cases. 282 80

The retrovirus-associated adult T-cell leukemia/lymphoma (ATL) has not been previously documented in Taiwan. Five cases identified recently by the authors are reported. Three of the patients were women, and their ages ranged from 36 to 60 years. The most important diagnostic clue was the observation of polylobated lymphoid cells in the peripheral blood. Other variably observed significant features included hypercalcemia, cutaneous eruptions, osteolytic bone lesion, hepatomegaly, and lymphadenopathy. Surface marker studies revealed that the leukemic or lymphoma cells were T-helper cells. Histopathologic examination revealed one case of pleomorphic type and three cases of medium-sized cell type. No tissue was available for study in one case. The diagnosis of ATL was confirmed by the indirect immunofluorescence test on MT-1 cell for antibodies to adult T-cell leukemia virus-associated antigen (ATLA). Three patients were dead within 6 months, and two patients had been in clinical remission for 7 and 10 months, respectively. These two latter cases were similar to the so-called smoldering type of ATL. Two descendents among nine relatives of the patients were also positive for anti-ATLA (22%). Two husbands were negative. Four of the five patients lived in the same county in northeastern coastal Taiwan, which suggested a possible clustering of ATL in that region.
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PMID:Adult T-cell leukemia/lymphoma in Taiwan. A clinicopathologic observation. 286 99


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