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)

An adult T cell leukemia (ATL) accompanied with Isospora belli infection was described. A 65-year-old male was admitted to our hospital because of a two month history of watery diarrhea. On admission, physical examination showed slight pallor but no detectable superficial lymphadenopathies. Hepatosplenomegaly was not observed. Laboratory examination revealed a leukocyte count 5,500/microliters with 10% abnormal lymphoid cells. A majority of the abnormal lymphoid cells expressed both CD 4 and CD 8 antigens. The patient was diagnosed as chronic ATL, since anti-HTLV-1 antibody in his serum and monoclonal integration of HTLV-1 proviral DNA in his peripheral mononuclear cells were detected. Isospora belli was found in his feces thereafter, and trimethoprim/sulfamethoxazole was effective for diarrhea. In Japan, there have been only 9 reported cases of lymphoproliferative disorders (including five ATL patients) accompanied with Isospora belli infection. From the descriptions in those reports, these 9 cases might all be ATL patients.
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PMID:[Isospora belli infection in a patient with adult T-cell leukemia]. 163 21

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.
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PMID:Assessment of abdominal involvement of adult T-cell leukemia/lymphoma by ultrasonography: comparison among four clinical types. 165 79

A 60-year-old woman was admitted to our hospital with symmetrical arthritis of the knees. During the 2 years preceding admission, she had experienced recurrent arthritis. A histological examination of her synovial tissue showed prominent villous proliferation of the synovial cells, prominent vascularity throughout and an inflammatory infiltrate composed of abnormal mononuclear cells. Three months later, she developed fever, skin eruptions, lymphadenopathy and hepatosplenomegaly. She also had hypercalcemia and there was abnormal lymphocytosis in her blood smears. She was diagnosed as having adult T cell leukemia. Parenteral chemotherapy treatment with adriamycin and cyclophosphamide gave remission of all the manifestations of disease, including arthritis. Her leukemia recurred, however, and she died 6 months after the diagnosis was made.
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PMID:A case of adult T cell leukemia complicated by proliferative synovitis. 202 26

An adult T cell leukemia associated with pure red cell aplasia-like lesion was described in this paper. A 51 year-old woman was admitted because of headache and palpitation in October 1988. On admission, physical examination showed marked pallor but no detectable superficial lymphadenopathies. Hepatosplenomegaly was not observed. The blood examination revealed normocytic anemia with Hb of 6.6 g/dl and marked leukocytosis of 18,800/microliters with 43% ATL cells. The bone marrow aspirate showed moderate infiltration of ATL cells and a few erythroblasts. The bone marrow biopsy disclosed moderate infiltration of ATL cells, only a few erythroblasts with maturation arrest and marked fibrosis. The erythropoietin in serum was elevated (686 IU/microliters). To clarify the mechanism of development of the PRCA-like lesion, the peripheral blood lymphocytes (ATL cells) or serum of the patient was added to in vitro erythroid colony formation. The patient's serum increased BFU-E but either serum or lymphocytes didn't inhibit the growth of CFU-E compared with control.
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PMID:[Adult T-cell leukemia associated with pure red cell aplasia-like lesion]. 212 22

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.
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PMID:Comparative study of cutaneous T-cell lymphoma and adult T-cell leukemia/lymphoma. Clinical, histopathologic, and immunohistochemical analyses. 224 93

A 53-year-old woman born in Kamo village of Shizuoka prefecture was admitted to Juntendo Izunagaoka hospital complaining cough and appetite loss. On physical examinations, general lymphadenopathy and hepatosplenomegaly were recognized. And also dry rales and wet rales were heard in the bilateral lungs. On hematological examinations, leukocytes has counted 74,900/microliters, of which 61% atypical lymphocytes, and there were HTLV-I infection and positive anti-ATLA antibody. By the above results, she was diagnosed as adult T cell leukemia (ATL). T cell subset analysis was (CD4-, CD8-, CD3-), which is rare in ATL. Three months after the admission, she was died of disturbances of respiratory function in spite of VEPA therapy. Surface marker changed from CD3- to CD3+ in the course. To illuminate this mechanism will be a key step for the future study.
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PMID:[Adult T cell leukemia with CD4- and CD8-]. 252 5

Adult T-cell leukemia-lymphoma, an aggressive T-cell leukemia, is characterized by the presence in the peripheral blood of malignant T cells that have highly indented or lobulated nuclei. Phenotypically the cells are usually helper T cells, but functionally they behave as suppressor cells. Patients have skin and lung involvement, hepatosplenomegaly, moderate lymphadenopathy sparing the mediastinum, and various metabolic abnormalities such as hypercalcemia. The clinical course may be chronic or acute, usually followed by a rapidly progressive terminal course. Adult T-cell leukemia-lymphoma is now known to be caused by human T-cell lymphotropic virus type I, which has been identified in the cells of patients with the disease.
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PMID:Adult T-cell leukemia-lymphoma. 266 14

A 58-year-old man, born in Nagasaki prefecture, was admitted to our hospital because of anorexia and general fatigue on November 22, 1984. Hepatosplenomegaly was found without skin eruption. The blood examination on admission revealed leukocytosis (50,800/microliter) and atypical lymphocytes with hyperlobulated nuclei. He had hypercalcemia, and hepatic and renal damage. A diagnosis of adult T cell leukemia (ATL) in the acute stage was made. Treatment with KM2210, a conjugate of chlorambucil and estradiol, was started, and his peripheral leukocytes decreased gradually reaching, 19,700/microliter by the end of this medication. His leukocyte count continued to decrease after discontinuation of KM2210 and reached a nadir of 4,700/microliter. Hepatosplenomegaly and hypercalcemia also improved. About one month later, recurrence of the disease occurred and he was again treated with KM2210. Although the second course of the KM2210 therapy was also successful in relieving hepatosplenomegaly and leukocytosis, it proved impossible to ameliorate his poor condition and he died of DIC. Our case suggests that KM2210 has a remarkable cytotoxic effect against ATL cells even in the acute stage but the optimal schedule of treatment with this new drug should be established in order to obtain more satisfactory therapeutic results.
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PMID:[A case of adult T cell leukemia treated with a new chemotherapeutic agent, KM2210]. 287 13

Adult T-cell leukemia (ATL) is characterized by peripheral lymph node enlargement, hepatosplenomegaly and skin lesions. The association of local mass lesions of other organs with ATL is extremely rare. This report describes a 57-year-old woman with chronic type ATL with associated local tumor masses in the nasal cavity, paranasal sinuses and larynx as well as skin infiltration. Histologic investigation of the skin lesion and nasal mucosa revealed non-Hodgkin lymphoma, diffuse, mixed type. Her chief complaints were progressive dyspnea and hoarseness. Leukemic cell masses in her upper respiratory tract caused narrowing of the airway, which was responsible for her complaints.
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PMID:Upper respiratory tract involvement in adult T-cell leukemia. 289 71

HTLV-I, the first human oncovirus, is a type C retrovirus linked to the development of ATLL. The virus shows a striking ethnogeographic distribution that is only partially understood. Certain populations at high risk for AIDS appear to have a higher incidence of HTLV-I infection. The extended latent period renders present knowledge of the sequelae and natural history of HTLV-I seropositivity incomplete, although recent data suggest that HTLV-I infection may have important implications for blood transfusion, organ transfer, and public health policy. A variety of clinical syndromes have been associated with infection, ranging from an asymptomatic carrier state to acute ATLL with lymphadenopathy, hepatosplenomegaly, hypercalcemia, cutaneous lesions, and systemic immunosuppression. Conventional chemotherapy is marginally effective; innovative approaches to therapy are presently being evaluated.
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PMID:Manifestations of human T-lymphotropic virus type I infection. 289 61


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