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)

We describe here a case of T-cell lymphocytic leukemia (T-CLL) which coexpressed CD4 and CD45RA cell-surface antigens and functioned as suppressor inducer cells. The patient, an 81 year-old man, had massive generalized lymphadenopathy. His hemoglobin was 9.4g/dl, the platelet count 94,000, and the WBC was 895,000/microliters with 98% abnormal lymphoid cells. He had massive hepatosplenomegaly. Serum LDH was elevated to 3,990 u/l. The T-CLL cells coexpressed antigens detected by MAbs CD2, CD3, CD4, CD5, Ti(TcR alpha/beta; WT31) CD45 and CD45RA, but did not express any other antigens including CD1, CD8, CD29, and TCR gamma/delta, Ti gamma A and TQ-1. The cell-surface phenotypes of the cultured cells established by utilizing recombinant interleukin 2 were basically the same as those of the uncultured peripheral blood lymphoid cells. Both the peripheral blood and cultured cells clearly showed gene rearrangement for T cell receptors, TcR beta and TcR gamma. No association with human T-cell leukemia virus-1 (HTLV-1) was found by means of electron microscopic studies or the application of MAbs to p19 and p24 of HTLV-1. No anti-HTLV-1 antibody was detected. By the means of two color fluorescence, it was clearly demonstrated that the leukemic cells possessing CD4 in the peripheral blood and cell cultures coexpressed CD45RA, but did not express either CD29 or TQ-1. In vitro immunoglobulin synthesis by normal T and B cells was remarkably reduced in the presence of CD8+ T and leukemic cells. This suggests suppressor inducer T cell activity for the leukemic cells.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:CD4+, CD45RA+, CD29- T-cell lymphocytic leukemia functioning as T suppressor inducer for B-cell immunoglobulin synthesis. 769 6

The J2E cell line is an immature erythroid line which terminally differentiates in response to erythropoietin (epo), producing mature, hemoglobin-synthesizing red blood cells. We have shown that when these cells were injected into mice a rapid and fatal erythroleukemia developed with symptoms of severe anemia and hepatosplenomegaly. Southern blotting demonstrated that the leukemic cells were the introduced J2E cells. In addition to spleen and liver, the bone marrow was a major site of leukemic cell infiltration, and when grown in vitro leukemic cells from bone marrow remained responsive to erythropoietin. We reasoned, therefore, that treatment of mice with this hormone should alleviate the erythroleukemia, but regular injections of epo in vivo failed to arrest the progress of the disease. However, when bone marrow from leukemic mice was exposed continuously to the hormone ex vivo, before reinfusion into naive recipients, a marked extension in life span was observed. It was concluded that ex vivo epo treatment could be used therapeutically for J2E cell erythroleukemias.
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PMID:A rapid fatal erythroleukemia caused by J2E cells can be treated ex vivo with erythropoietin. 776 54

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease, whose origin seems to lie in a acquired defect in the membrane of the pluri-potential hematopoietic cell. Chronic or intermittent acute hemolytic syndrome is the most frequent clinical manifestation, although in the literature there are also some references to the leukocytic and immunologic disorders of this disease. In this paper, we present the case of a 63-year-old patient with NPH who developed severe neutropenia and sustained febrile syndrome. In the past four years, she had suffered frequent episodes of fever and leukopenia, which apparently disappeared spontaneously. In the physical exploration, we observed hepatosplenomegaly. The hemogram showed mild iron deficiency anemia (hemoglobin 10.8 g/dl), severe neutropenia (neutrophil 0.3 x 10(9)/l) and significant reticulocytosis (610 x 10(9)/l). Iron deposits were greatly reduced in the marrow. Simultaneously to a new febrile episode and isolation of Escherichia coli in the urine, there was a severe anemization (hemoglobin 5 g/dl) and a significant thrombopenia (platelets 30 x 10(9)) resulting in a positive hemosiderinuria and sucrose test. The study of the leukocytic function showed a defect in the neutrophil chemotaxis, although a normal phagocytic capacity and microbicidal activity. In the following nine months, the patient had several severe infections, with intense but transitory pancytopenia, which always improved when treating the infection with antibiotics. The patient died due to a septic shock twelve months after the diagnosis. Recurrent febrile episodes and severe neutropenia are very rare in the PNH (less than 4% of the cases). The cause of these disorders is still unknown.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Recurrent infections, severe neutropenia and neutrophil chemotaxis defect in paroxysmal nocturnal hemoglobinuria]. 786 56

Nitrobenzene (NBZ) is primarily employed as an oxidizing agent in the synthesis of analine and benzene compounds. It produces myelotoxic effects and effects on erythrocytes in both animal models and man. Reported hepatosplenomegaly and effects on the bone marrow are indicators that NBZ may be immunotoxic. In these studies, female B6C3F1 mice were exposed to 30, 100 and 300 mg/kg of NBZ in corn oil by gavage for 14 consecutive days. To assess the immunotoxic potential of NBZ, body and organ weights were determined and selected immunologic and host resistance responses were studied. In these studies, the liver and spleen appeared to be the primary target organs. Both liver and spleen weights were dose dependently increased. Gross histopathologic examinations revealed significant changes in the spleen, consisting of severe congestion of the red pulp areas with erythrocytes and reticulocytes. Serum chemistry profiles showed increases in alanine aminotransferase and aspartate aminotransferase activities, indicating liver toxicity. Hematologic studies showed a decrease in erythrocyte number and a concomitant increase in mean corpuscular hemoglobin and mean corpuscular volume. A dose-dependent increase in peripheral reticulocytes was also seen. DNA synthesis was enhanced, as was the number of formed elements and the number of monocyte/granulocyte stem cells in the bone marrow of treated mice. IgM responses were decreased and the phagocytic activity of macrophages in the liver was dose dependently increased with a concomitant decrease in the activities in the spleen and lung. Other immunological parameters examined were unchanged. Host resistance to microbial or viral infection was not markedly altered by NBZ; however, there were trends towards increased susceptibility where T-cell function contributes to host defense. These data indicate that NBZ-induced hemolysis and liver injury are linked to the observed alterations in bone marrow activity.
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PMID:Immunotoxicity of nitrobenzene in female B6C3F1 mice. 798 85

Ten children (five boys and five girls) with juvenile chronic myelocytic leukemia were seen over a period of 12 years (1980-1991) at the All India Institute of Medical Sciences, New Delhi. With the exception of one who was aged 4.5 years, all children were below 4 years of age (mean age 20.4 months). The presenting features included fever, bleeding secondary to thrombocytopenia, marked hepatosplenomegaly, and skin rash. The striking hematological features were anemia, thrombocytopenia, peripheral blood monocytosis, and normoblastemia. There was no significant myeloid proliferation in the bone marrow aspirate (mean M:E = 5:1), while erythroid proliferation was prominent along with monocytosis (mean 11.2%). Fetal hemoglobin was raised in 8 of the 10 patients (mean 14.1%). Long-term survival was poor, with maximum survival being 18 months in one case. New modalities of management of this rare entity are discussed.
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PMID:Juvenile chronic myelocytic leukemia--report of 10 cases. 799 Jul 56

Gaucher disease is a sphingolipid storage disorder caused by a deficiency of the lysosomal enzyme glucocerebrosidase (GC) and the consequent deposition of glucocerebrosides into the cells of the macrophagic system. Among the three types of clinical disease, type 1 leads to hepatosplenomegaly, hypersplenism and skeletal abnormalities including bone pain, osteopenia and fractures. Two pediatric female patients with moderately severe type 1 Gaucher disease were treated with commercially available GC, mannose terminated to be macrophage-targeted. GC was given by intravenous infusion (30 to 60 units per kilogram of body weight every two weeks) for 8 and 18 months. The hemoglobin concentration increased and the serum acid phosphatase decreased in both patients. In the most affected child, hepatic volume decreased significantly and bony symptoms disappeared. Infusions were uneventful except for an episode of anaphylaxis that subsided rapidly, allowed resumption and did not affect efficacy. These observations are in agreement with the international experience in approximately 800 cases, with good tolerance in all type 1 patients who show objective clinical improvement; patterns of response are variable from patient to patient, independent from previous splenectomy, and dose-dependent; the dose can be tapered after a period of time. Antibodies anti-GC are seen in 13% of the patients, but their presence does not have clinical consequences. The cost of the enzyme makes it crucial to define precise indications, optimal dosing schedules, duration of treatment and cost-benefit ratio.
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PMID:[Enzyme replacement therapy in type 1 Gaucher's disease]. 799 30

We report a rare case of idiopathic myelofibrosis transformed to acute myelomonocytic leukemia associated with non-Hodgkin's lymphoma. A 64-year-old woman was admitted to our department because of anemia and leukocytosis. On admission, anemia and hepatosplenomegaly were noted. The hemoglobin content was 6.8 g/dl, and WBC count was 26,200/microliters with an increased number of immature neutrophils. Bone marrow biopsy revealed an increased amount of reticulin fiber. Because she had no disease which causes secondary myelofibrosis, idiopathic myelofibrosis was diagnosed, and she was treated with prednisolone, anabolic steroid and blood transfusion. Fifteen months after the diagnosis of myelofibrosis, blast increased in her peripheral blood and her spleen and liver enlarged remarkably. A tumor of right parotid region was recognized at the same time. The pathological diagnosis of biopsied tumor was non-Hodgkin's lymphoma. The cytochemical study of blasts in her peripheral blood showed that she had acute myelomonocytic leukemia. In spite of intensive chemotherapy, she died from heart failure, respiratory failure and renal insufficiency.
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PMID:[Idiopathic myelofibrosis transformed to acute myelomonocytic leukemia associated with non-Hodgkin's lymphoma]. 807 93

We report a 24-year-old woman who had acute monoblastic leukemia associated with t(16;21) (p11;q22). She was referred to our hospital in April 1992 because of high fever and hemorrhagic diathesis. Physical examination on admission showed no hepatosplenomegaly. The hemoglobin was 5.1g/dl, platelet count 1.7 x 10(4)/microliters, the white blood cell count 18,700/microliters. Bone marrow aspirate showed that 86% of nucleated cells were monoblasts which were positive for peroxidase and alpha-naphtyl butyrate esterase. She was diagnosed as having M5a. Dysmegakaryopoiesis, such as micromegakaryocytes and megakaryocytes with multiple small separated nuclei, was seen in the bone marrow. Chromosomal analysis revealed t(16;21). Complete remission was achieved after two courses of BHAC-DMP therapy, but dysmegakaryopoietic features remained. She relapsed in September 1992. Review of the literature and this patient indicate that acute nonlymphocytic leukemia with t(16;21) is associated with multilineage leukemic differentiation.
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PMID:[Acute monoblastic leukemia (M5a) with dysmegakaryocytopoiesis associated with t(16;21) (p11;q22)]. 813 14

We investigated whether recombinant alpha 2b interferon (r alpha 2bIFN) would reduce the proportion of bone marrow Philadelphia chromosome (Ph) cells in chronic-phase chronic myelogenous leukemia (CML) by treating 107 previously untreated patients daily with r alpha 2bIFN at 5 x 10(6)IU/m2 subcutaneously. Patients with complete remission, partial remission, or partial hematologic remission received treatment until progression; those with progressive disease were taken off study and observed for survival. Sixty-three (59%) of the patients achieved at least a partial hematologic remission (24 complete remissions and 39 partial remissions). The median time to response for the 63 responders was 3.4 months, with a median duration of remission of 52 months and with 81% of responders continuing in remission beyond 12 months. The median survival for the 107 patients was 66 months. Of 78 patients with cytogenetic follow-up data, 31 (40%) achieved a partial cytogenetic response (n = 17) or a complete cytogenetic response (n = 14). The percentage of cytogenetic responders among all patients was 29% (31 of 107 patients). The median time to first cytogenetic response was 9 months. A major dose reduction of r alpha 2bIFN (> or = 50%) was required at some time during treatment in 38% of patients, 26% required 10% to 49% dose reductions, and 36% had minor dose reductions of < or = 10%. No association was observed between dose received and the attainment of a cytogenetic response. None of the usual prognostic factors (sex, race, performance status, weight loss, time from diagnosis to treatment, hepatosplenomegaly, age, symptoms, hemoglobin, or platelet, blast, basophil, or white blood cell count) were significantly related to survival. These data provide confirmation that major cytogenetic responses to prolonged administration of subcutaneous r alpha 2bIFN occur in 20% to 38% (95% confidence interval) of chronic-phase Ph-positive patients. Although it is hypothesized that patients achieving major cytogenetic responses to r alpha 2bIFN should have prolonged remission duration and survival compared with nonresponders, analyses of the effect of cytogenetic responders by both "landmark" and time-dependent covariate techniques fail to provide statistically significant evidence for an effect of cytogenetic response on remission duration or survival. This may be due in part to an effect size insufficiently large to be detected with the number of patients treated in this study. Thus, confirmation of remission duration or survival benefit, if any, of r alpha 2bIFN therapy in Ph-positive chronic-phase CML must await the outcome of randomized trials comparing IFN with conventional agents.
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PMID:Prolonged subcutaneous administration of recombinant alpha 2b interferon in patients with previously untreated Philadelphia chromosome-positive chronic-phase chronic myelogenous leukemia: effect on remission duration and survival: Cancer and Leukemia Group B study 8583. 811 47

The paper summarizes the results of clinical examinations of imported malaria in 48 patients, semi-immune foreigners infected with P. falciparum and P. vivax/P. ovale. Of the objective manifestations, hepatosplenomegaly was predominant in P. falciparum infections (34%), whereas in P. vivax infections its occurrence rate was 19%. Body temperature over 38 degrees C with shivering had a higher frequency in P. vivax (81%) compared to P. falciparum (41%) infections. Pathologically altered hematologic values, particularly mild to medium increase in sedimentation rate, were found in all patients with P. vivax/P. ovale, while in patients infected with P. falciparum only in those whose parasitemia exceeded 10,000 trophozoites in 1 microliter blood. Reduced values of hemoglobin, hematocrit, and mean erythrocyte volume are indicative of microcytic anemia of mild degree. Biochemical examinations yielded slightly increased values of the thymol turbidity test and mild reduction of serum proteins at simultaneous increase of albumins (8% of the patients examined). Of the parameters of humoral immunity, there was a rise in immunoglobulin values concerning particularly IgG and IgM, and that in all types of malarial infection studied. Increased values of circulating immunocomplexes had a high frequency rate with all plasmodial species, in P. falciparum infections independently of the degree of parasitemia. (Tab. 7, Ref. 13.)
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PMID:[The clinical picture of imported malaria and its relation to P. falciparum parasitemia]. 835 68


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