Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adult T-cell leukemia/lymphoma (ATLL) is an HTLV-I associated lymphoid malignancy frequently seen in Japan. Abdominal involvement in 40 patients with ATLL were assessed by ultrasonography and the findings seen in four clinical types, acute, chronic, lymphoma and smoldering, were compared. Splenomegaly was frequently found in the cases of acute and lymphoma types, and the sizes of the spleens measured by ultrasonography correlated well with the disease activity. Hepatomegaly was also found more frequently in acute and lymphoma types, and hepatosplenomegaly was proved to be due to the infiltration by ATL cells. Nodular lesions in spleen and liver and abdominal lymph node swelling were also found frequently in the lymphoma type but rarely in the other types. Ascites, pleural effusion, and pericardial effusion were found in the active stage of acute and lymphoma types. Ultransonography also could detect findings associated with therapies. Thus, ultrasonography studies were found to be very useful for assessing the clinical classification, examining various pathological conditions associated with ATLL, and monitoring the disease activity.
 ...
PMID:Assessment of abdominal involvement of adult T-cell leukemia/lymphoma by ultrasonography: comparison among four clinical types. 165 79

A 44-year-old Aborigine with Adult T-cell Leukaemia/Lymphoma (ATLL) due to HTLV-I is reported. He presented with transverse myelitis of subacute onset, and subsequently developed frank T-cell leukaemia complicated by splenomegaly and hypercalcaemia. Cell surface marker studies showed a phenotype of CD3+ CD4+ CD8- CD25+, and serological and molecular studies confirmed HTLV-I infection. This is the first report of ATLL in an Australian Aborigine.
 ...
PMID:Adult T-cell leukaemia lymphoma in an aborigine. 175 23

A 39-year-old woman was first admitted to our hospital with increased white cell count on May, 1983. Physical examination showed only mild splenomegaly. Hematological examination revealed leukocytosis (14,600/microliters) with ATL cells (59%). Serum anti-HTLV-I antibody was positive. Examination of HTLV-I provirus in the abnormal T cells revealed the defective type. She was diagnosed as chronic type of ATL based on the clinical features. Cytogenetic study of the ATL cells revealed 47, xx, +4. For 12 months, she was followed without any therapy. WBC reduced to almost normal range and ATL cells decreased to 3 to 6% for 8 months. On May, 1985, she was readmitted to our hospital because of leukocytosis (32,200/microliters), and increased ATL cells (57%). She was diagnosed as crisis of ATL. Investigation of the proviral DNA and chromosome showed the same results as those of the chronic phase, indicating that ATL cells in both the chronic phase and the crisis phase originated from the same clone. She died after 3 months from massive diarrhea. Postmortem examination showed the extensive infiltration of leukemic cell in the small intestine.
 ...
PMID:[A case of adult T-cell leukemia with a defective HTLV-I proviral DNA, in which the single T-cell clone appeared to have progressed from chronic phase to crisis]. 192 Aug 41

A case of acute adult T-cell leukemia-lymphoma (ATLL) was observed in northeast Italy, presenting with fever, lymphadenomegaly, splenomegaly, hypercalcemia and renal failure. Leukaemic cells were morphologically typical, expressed a T-cell CD4+ phenotype, did not display any helper functions, and grew in vitro under supply of exogenous interleukin-2. Antibodies to human T-cell lymphotropic virus (HTLV-I) were found in the serum, and the virus was isolated from leukaemic cells. The family members who could be tested were seronegative. The patient had never travelled outside Italy, had never received blood transfusions and did not belong to any known categories at risk of viral disease transmission. Present knowledge of the epidemiology of HTLV-I infection warns that other cases of HTLV-I induced disease are expected to occur outside already recognised endemic areas. This case suggests that untraceable, presumably short-term exposures can also account for HTLV-I transmission.
 ...
PMID:HTLV-I positive adult T-cell leukaemia-lymphoma: report of a typical case from Italy. 198 Apr 80

A case-control study was performed in Martinique, French West Indies, comparing 66 anti-p24 antibody carriers to 91 seronegative subjects for HTLV-I, matched for age and place of residence. The aim of our study was to identify factors associated with HTLV-I infection and to observe whether clinical examination and biological measurements would reveal any abnormalities among the seropositive subjects. We observed a predominance of females among seropositive subjects (74% compared to 59%, p less than 0.05), and a greater risk due to earlier blood transfusions (p less than 0.001). This survey revealed important differences between cases and controls regarding socioeconomic factors: cases had fewer luxuries or advantages (i.e. bathroom, toilets, refrigerator, telephone, p less than 0.01), were more corpulent (p less than 0.05), and more often widowed, divorced or separated (p less than 0.01) than the controls. Although the differences were not significant, the seropositive donors seemed to be less educated, and were from a lower socioprofessional class than the seronegative donors. With regard to clinical symptoms (infections, adenopathies, splenomegaly, hepatomegaly) and biological parameters (blood count; T-cell subsets, electrophoresis of protids, immunoglobulins, calcemia, antischistosomal antibody), seropositive subjects appeared to be healthy; no parameters, except for alpha 1 globulin (p less than 0.05) and monocytes (p less than 0.05), were found to be correlated with seropositivity; but these two parameters remained within their normal ranges. This study confirms blood transfusion as a risk factor. It underscored the importance of socioeconomic factors for seropositivity.
 ...
PMID:HTLV-I infection in French West Indies: a case-control study. 204 16

We describe the clinical, ultrastructural, and immunophenotypical characteristics of four cases of an unusual type of T cell leukemia. Clinical features included high WBC, ranging from 26-148 x 10(9)/liter, bone marrow infiltration, splenomegaly, and lymphadenopathy. Skin involvement was not documented at presentation, but it was seen as a terminal event in one patient with a pattern of dermal lymphocytic infiltration different from that usually seen in Sezary syndrome. By ultrastructural analysis, the circulating lymphoid cells were indistinguishable from small Sezary cells in two cases, resembled large Sezary cells in one case, and consisted of a mixture of small Sezary cells and prolymphocytes in the remaining case. The cells from all cases had a mature T cell phenotype, TdT-, CD1a-, CD2+/-, CD3+, CD5+. In addition, the cells were either CD8+, CD4- or CD8+, CD4+ or CD4-, CD8-; and, in only one case, the findings were similar to those of Sezary syndrome cells: CD4+, CD8-, CD7-, BE-2+. In the latter case, serological and immunological assays were positive for HTLV-I while these were negative in two other patients investigated. The features of these patients suggest that Sezary cell leukemia is a distinct clinico-pathological entity although the alternative diagnosis of adult T cell leukemia/lymphoma could not be excluded in the HTLV-I+ case. Sezary cell leukemia appears to be resistant to current chemotherapy regimens and is associated with an aggressive clinical course and short survival.
 ...
PMID:Sezary cell-like leukemia: a distinct type of mature T cell malignancy. 236 82

The prevalence of antibodies against HTLV-III and -I was studied among populations of 6 distinctly different regions of Kenya, an equatorial African country in which AIDS has rarely been observed. Overall, 21% of subjects had ELISA reactions suggesting the presence of antibody against HTLV-III. The frequency of HTLV-III antibodies was highest among the Turkana people (50%) and lowest among the Masai (8%). Prevalence increased with age but was not related to sex. The pattern of ELISA-detected antibody against HTLV-I was similar. The specificity of these antibodies was supported by Western blot analysis of a subset of sera with high and low ELISA ratios, in which 66% and 73% of those with ELISA ratios considered positive (= greater than 5.0 in this study) also had a profile of bands consistent with HTLV-III and HTLV-I respectively. The antibodies detected were not cross-reactive between HTLV-III and HTLV-I on Western blot analysis. In a series of subjects with various parasitic and infectious diseases, patients with idiopathic splenomegaly and with schistosomiasis had a high proportion of antibodies against both HTLV-III and HTLV-I. This survey shows that reactivity in the ELISA HTLV-III and HTLV-I assays are common among Kenyans but vary considerably by region.
 ...
PMID:Regional variation in prevalence of antibody against human T-lymphotropic virus types I and III in Kenya, East Africa. 298 91

LGL leukemia results from a chronic, clonal proliferation of LGL. Chronic neutropenia with recurrent bacterial infection and splenomegaly are common clinical manifestations. Rheumatoid arthritis coexists in some of these patients, who thus resemble patients with Felty syndrome. Other hematologic abnormalities that may occur include pure red-cell aplasia and adult-onset cyclic neutropenia. Lymphoid infiltration of bone marrow, splenic red pulp cords, and hepatic sinusoids is characteristic; lymph node and skin involvement are rare. Multiple serologic abnormalities are frequently present, including positive tests for rheumatoid factor and/or antinuclear antibody, polyclonal hypergammaglobulinemia, and circulating immune complexes. Antineutrophil and antiplatelet antibodies are often present. Leukemic LGL exhibit phenotypic heterogeneity; the most common phenotype in our patients is CD2+, CD3+, CD8+, HNK-1+, CD16-. Despite markedly increased numbers of LGL, functional activity of the cells is usually decreased. The mechanism of cytopenias is uncertain: in pure red-cell aplasia, it appears to be due to suppressive effect on erythropoiesis by abnormal LGL, but in patients with chronic neutropenia it may be antibody-mediated. Although most patients appear to have a relatively benign clinical course, mortality from infections and progressive lymphoproliferation is substantial. Optimal therapy remains undefined. Some preliminary evidence suggests that LGL leukemia may be associated with infection with a retrovirus similar to HTLV-I. Although relatively rare, LGL leukemia is of interest because a better understanding of this disease process may contribute to our knowledge of autoimmune diseases, the immunoregulatory functions of LGL, and the mechanisms controlling normal hematopoiesis.
 ...
PMID:Large granular lymphocyte leukemia. Report of 38 cases and review of the literature. 362 48

A case of a 20-years-old black man from Salvador, Bahia with HTLV-I associated T cell lymphoma is presented. In spite of the absence of splenomegaly and leukemia, the patient had a marked cephalic tumoral infiltration associated with axillary tumors in a pattern not yet described in adult T cell lymphoma. Peripheral blood involvement was observed later on in the course of the disease. The patient underwent chemotherapy but died seven months after diagnosis.
 ...
PMID:HTLV-I associated cutaneous T-cell lymphoma--report of a case with atypical clinical presentation. 752 65

Transgenic mice that carry the HTLV-I Tax gene develop an exocrinopathy with some similarities to Sjoegren's syndrome. Our experiments reveal that these mice have lymphadenopathy and splenomegaly composed primarily of B lymphocytes, as well as abnormal levels of secreted immunoglobulins. To gain insight into whether the lymphadenopathy manifested by these transgenic mice was the result of induction of cytokines by Tax, we utilized cell lines from these mice to study in vitro B-cell responses. Conditioned media (CM) derived from the cell lines caused B-cells to proliferate when a second signal, surface Ig cross-linking, was provided. The CM also caused a marked enhancement of IgM secretion by spleen cells or by purified B-cells treated with supplemental cytokines. The B-cell proliferative response and enhanced IgM secretion have not been attributed to a known cytokine. These results suggest that the CM from the cell lines contain a factor(s) involved in novel pathways of B-cell growth and differentiation that may participate in the pathologic development of autoimmune disease.
 ...
PMID:Abnormal B-cell function in HTLV-I-tax transgenic mice. 770 Jun 28


1 2 Next >>