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

A 43 year-old man admitted to our hospital because of fever and splenomegaly. Laboratory findings were as follows: Hb 9.5 g/dl, Plts 4.9 X 10(4)/microliter, LDH 2,348 IU/l. Bone marrow findings showed tumor cell 47% with or without phagocytosis. The tumor cells were stained positive lysozyme and alpha 1 antitrypsin. Cytogenetic study was 47, XY, -7, -8, +9, -11, -12, -19, -21, 3q+, 6p+, +6 markers. This case was diagnosed as malignant histiocytosis. Complete remission was achieved with CHOP-E chemotherapy. Remission has been maintained with repeated this therapy. Etoposide deserves a good evaluation in the treatment of malignant histiocytosis. Some cases of malignant histiocytosis with a t(2; 5) (p23; q35) translocation were often reported in Europe and America, while there was no specific chromosomal abnormalities with malignant histiocytosis in Japan.
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PMID:[Malignant histiocytosis with complex chromosomal abnormality: successful treatment with CHOP-E chemotherapy]. 204 Nov 66

An 18 years old female was admitted to hospital due to pancytopenia on May 25, 1987 and found to have petechiae, mild hepatomegaly and severe splenomegaly. The bone marrow was found to contain 12% of blast cells. Splenectomy was performed followed by CHOP therapy. In September, 1987 the peripheral blood was found to contain tumor cells, which turned out to be resistant to various combination chemotherapies. The patient died on August 21, 1988. The phenotype of tumor cells in this case was CD2+ CD7+ CD3+ CD4- CD8- WT31-. Genetic analysis detected rearrangement of the beta and gamma chain of TcR but not transcription or translation of the beta chain of TcR, while the antibodies of delta TCS 1 and TcR delta 1 to the delta chain of TcR were positive. From this fact, the present case was considered to be the malignant counterpart of normal CD3+ WT31- double negative T cells. The reactivity of this tumor cells to IL-2 and IL-1 beta suggested the association of the IL-2R beta chain.
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PMID:[T gamma lymphoma with CD3+ CD4- CD8- WT31- and TcR gamma delta]. 213 75

The diagnostic findings of malignant histiocytosis (MH) were analysed in 12 consecutive patients in a single institution. Most patients presented with systemic symptoms and lymphadenopathy (92%), splenomegaly (100%) and hepatomegaly (67%). Neurologic symptoms were present in three patients, while involvement of other organs was present in five patients. The incidence of severe thrombocytopenia was 92% of anaemia 92% and of leucocytopenia 67%. Serum angiotensin converting enzyme, alpha 1-antitrypsin and lysozyme were independently increased in 6/9, 3/10 and 1/9 patients respectively. High serum levels of tumour necrosis factor (TNF) were present in 3/10 patients, while serum levels of interleukin-1 were normal in 10/10 patients. Histologic evidence of MH was obtained in all patients by repeated biopsies of involved tissues. Four patients died prior to treatment. Seven patients were treated with combination chemotherapy, consisting of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) or MOPP (chloromethine, vincristine, procarbazine, prednisone), in some cases followed by non-cross-resistant second line chemotherapy, if no complete response was attained. The response rate of treated patients was 57%, and progression was observed in two patients. The median duration of response was 38 months. Three patients are alive without evidence of disease and off therapy (30+, 83+, 85+ months). Although MH is a potentially lethal disease, combination chemotherapy may offer a chance for cure in some patients.
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PMID:Clinicopathological diagnosis and treatment of malignant histiocytosis. 220 2

The clinical course of two patients with prolymphocytic leukemia of the suppressor T-cell phenotype is presented. Diagnosis was established by specific monoclonal antibody tests, cytochemical, ultrastructural and cytogenetic studies. The bone marrow showed a diffuse infiltration with medium sized lymphocytes displaying a prominent nucleolus. Both patients presented with pronounced splenomegaly, diffuse lymphnode involvement, leukocytosis, thrombocytopenia and anemia. One of them was refractory to chemotherapy using CHOP, Asparaginase, IMVP16, and irradiation to the spleen. He benefited from prednimustin and repeated leukaphereses, without reaching complete remission (C.R.). The second patient achieved a C.R. of 4 months duration with CHOP-Bleomycin. Survival was 16 and 11 months respectively.
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PMID:Prolymphocytic leukemia with suppressor T-cell phenotype: report of two cases. 252 11

Clinical and histopathologic material from 42 patients with peripheral T-cell lymphoma (PTCL) was reviewed. The median age was 63.5 years (range, 11-97 years). The male:female ratio was 2.8:1. Prior immune or lymphoproliferative diseases occurred in 36% of the patients. PTCL was advanced at presentation with B symptoms (67%), generalized adenopathy (69%), and stage III/IV disease (79%). Suspected lung or pleural involvement (21%), hepatomegaly (29%), and splenomegaly (43%) were common; marrow involvement was documented in 37% of the patients at presentation and in 51% of patients during the illness. Hypercalcemia and eosinophilia occurred in 19% and 29% of patients, respectively. Among patients receiving combination chemotherapy (BCOP, CHOP, BACOP, COMLA), eight (24%) of 33 achieved a complete remission and only four (12%) of 33 had a sustained complete remission. The median survival for PTCL was 11 months. Because of the poor response to standard therapy, clinical trials should identify cases of PTCL and evaluate newer regimens in this subset of aggressive lymphoma.
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PMID:Peripheral T-cell lymphoma: a clinicopathologic study of 42 cases. 637 20

Clinical and morphologic characteristics of hairy cell leukemia at 11 patients have been analyzed. The frequency of this disorder is 2.5% from all leukemias. This disorder is often retrospectively diagnosed--at 7 cases from our study. the disorder is often retrospectively diagnosed--older age (78%). Splenomegaly is the main clinical manifestation, at all 11 patients. Pancytopenia is frequent finding but at 4 patients the leucocyte count was over 10 X 10(9)/1 in the beginning. Hairy cells, although not always with typical appearance, in 90% of the cases are found in peripheral blood over 10%. Bone marrow biopsy cytological and hystological findings at 9 patients were typical for diagnosis. Fibrosis was present in 6 specimens. At 7 patients diagnosis was confirmed with histological examinations of lymphocyte concentrates from peripheral blood on thin and ultrathin sections, as well as with electron microscopy characteristic appearance of hairy cells. Pneumonia as complication was registered in 24 occasions, gastro-intestinal infections at 9, haemorrhagic syndrome at 4 and skin carcinoma at 2 cases. Treatment was variable--2 patients were observed for more than 50 months, 2 were splenectomised, of which one with complete remission longer than a year, while from 8 treated with COP protocol, complete remission was obtained in 5 (62%) patients, and two treated with CHOP protocol entered complete remission for longer than 12 months. Average survival is 51 months (2--144). Three (28%) patients died.
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PMID:[Hairy cell leukemia. Personal experience with 11 cases]. 668 Mar 1

A 60-year-old female was admitted complaining of anemia. We diagnosed her hereditary spherocytosis (HS) from spherocytosis and family history and found a tumor in her enlarged spleen. Splenectomy was performed and swollen paraaortic lymph nodes were found at laparotomy. The tumor in the spleen was diagnosed as Non-Hodgkin's lymphoma (follicular mixed type). After CHOP therapy she entered complete remission. Though the relationship between HS and malignant lymphoma was not clear, splenomegaly due to hemolysis inducing chronic stimulation might have resulted in malignant lymphoma.
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PMID:[Hereditary spherocytosis associated with non-Hodgkin's lymphoma in the spleen]. 796 56

The variable course of the disease, the advanced age of most patients and the absence of uniform criteria to evaluate treatment have constituted important setbacks in the therapy of CLL. The advent of clinical staging systems, which allow the identification of patients with different risks and the planning of appropriate therapy, constitutes a major advance. Results of trials based on these staging systems have demonstrated that treatment of patients with CLL in early stage is of no benefit and may even be harmful. By contrast, there is general agreement that patients in advanced stage should be treated. Chlorambucil, either daily or intermittently, and given alone or with corticosteroids, remains the most commonly used drug. Other single agents used in CLL include prednisone, busulphan and cyclophosphamide. Results are often comparable with those observed with chlorambucil alone, although sometimes with more toxicity. The benefit in survival terms of all these drugs remains to be proved. New agents, such as fludarabine, 2-deoxycoformycin and 2-chlorodeoxyadenosine, offer promise. However, the superiority of these drugs over chlorambucil needs to be demonstrated in randomized trials. Most combination therapy regimens have failed to show advantage over chlorambucil with or without prednisone when compared in clinical trials. In a previous randomized trial, the French Co-operative Group on CLL showed a beneficial role for low-dose adriamycin given with cyclophosphamide, vincristine and prednisone (mini-CHOP) in patients with stage C disease. However, these results were obtained in a small series of patients and need to be confirmed. Splenectomy can be considered in patients with autoimmune haemolytic anaemia or thrombocytopenia, with splenic destruction. Radiotherapy, administered either as 32P, total body irradiation, extracorporeal irradiation of blood or thymic irradiation, is effective in a few patients, but severe myelosuppression is a frequent sequel. Splenic irradiation has more often been used when splenectomy was difficult in the case of massive splenomegaly or immune cytopenia. New strategies, including the use of biological response modifiers (interferons, interleukins 2 and 4, erythropoietin, cyclosporine and monoclonal antibodies either alone or conjugated with immunotoxins), are presently under study. So far, only transient effects have been observed. Although complete remission in classical terms is frequently observed with these therapies, clonal remission is a very rare event and cure cannot be achieved.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Treatment of chronic lymphocytic leukaemia. French Co-operative Group on CLL. 803 94

A 59-year-old man was admitted in July, 1985 because of generalized lymphadenopathy and hypercalcemia. The biopsy specimen of the left inguinal lymph node showed diffuse large cell lymphoma. He achieved a complete remission (CR) in July, 1986 by CHOP therapy. In January, 1992, he visited the outpatient clinic with splenomegaly, and swelling of the left axillary and left supraclavicular lymph nodes. The biopsy specimen of the axillary lymph node showed follicular small cleaved cell lymphoma. Because the spleen and the lymph nodes regressed after the biopsy, he has been followed up without any specific treatment for lymphoma. Histologic change from low-grade lymphoma into more aggressive histologic pattern is well documented. However the converse phenomenon, occurrence of low-grade lymphoma after intermediate-, or high-grade lymphoma has rarely been reported. Histological examination is mandatory when a patient with diffuse large cell lymphoma is suspected to have relapsing disease after the long-term CR.
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PMID:[Occurrence of follicular small cleaved cell lymphoma following chemotherapy for diffuse large cell lymphoma]. 806 20

A 58-year-old woman complicated with rheumatoid arthritis (RA) was admitted to our hospital with right axillar lymphadenopathy and splenomegaly in November 1992. She was diagnosed as an anaplastic large-cell lymphoma (Ki-1 +) (stage IIIB) on the histological findings of the right axillar lymph nodes. She was treated with 11 courses of CHOP regimen between February 1992 and May 1993, and with mitoxantrone, etoposide (VP-16) and predonisolone in April 1992 and May 1993. The right axillar lymph nodes and spleen were irradiated at a dose of 36Gy in October 1992 and May 1993 respectively. In May 1993, peripheral blood showed WBC 89,000/microliter with 96% myeloblasts, Hb 8.3 g/dl, and Plt 124,000/microliter. Bone marrow aspirate revealed hypercellularity with 90% myeloblasts, which were positive for CD13 and HLA-DR. She was diagnosed as AML (M1). The karyotype showed normal. Southern blot analysis did not reveal the rearrangement of the MLL gene. She received the BHAC-DMP regimen and obtained complete remission. However, she relapsed during consolidation therapy, and died of cerebral bleeding. An autopsy revealed absence of a residual tumor. The mean interval from exposure to alkylating agent to the onset of secondary leukemia has been reported to be about 5 years, in contrast to a shortened interval of about 2 years for VP-16-induced leukemia. In our patient, it took only 1 year to have AML following chemotherapy for Ki-1 lymphoma. This suggests that her AML might be induced not only by treatments for RA and Ki-1 lymphoma, but also by immunological background such as RA.
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PMID:[Acute myeloid leukemia (M1) following chemotherapy for Ki-1 lymphoma complicated with rheumatoid arthritis]. 858 73


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