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 75-year old man was admitted to our hospital by complaining of his left supraclavicular lymphadenopathy. Physical examination also revealed splenomegaly and cervical lymphadenopathy. An abdominal CT scan showed paraaortic lymphadenopathy and splenomegaly with multiple low density area. A pathological diagnosis of Ki-1 lymphoma was made by the biopsied supraclavicular lymph node because the node consists of large cells with clear cytoplasms and conspicuous nucleoli and large cells were positive for IgM, lambda, CD20 and CD30 (Ki-1). The primary lesion of lymphoma was suspected to be spleen because of the radiological finding of the spleen. The patient responded to systemic chemotherapy (CHOP) with complete remission. Pathological and clinical characteristics of this B-cell Ki-1 lymphoma were discussed.
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PMID:[Splenic lymphoma of Ki-1 positive B-cell type treated with CHOP]. 131 79

This report describes the clinical spectrum and outcome of the hemophagocytic syndrome (HS) in 5 HIV infected patients. All 5 patients presented with fever, hepatomegaly and/or splenomegaly, confusion or coma and respiratory symptoms. Severe anemia was associated with thrombocytopenia and with neutropenia in 4 cases. Diffuse intravascular coagulopathy was present in 2 cases. Liver function tests were abnormal in three patients. The diagnosis of HS was made 2 to 12 weeks after the onset of symptoms and required in most patients repeated examinations of the bone-marrow, showing infiltration by histiocytes with prominent phagocytosis of blood cells. In one case this infiltration was not seen in the bone-marrow but only in the liver and the spleen. Varicella, mycobacterium infection, oesophageal candidiasis, Kaposi sarcoma were observed in the evolution of 3 patients. Anaplastic large cell Ki-1 lymphoma was present in one case. Four patients died as a result of complications of HS. The one patient with lymphoma survived.
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PMID:[Hemophagocytic syndrome in HIV infection]. 824 41

A total of 41 non-cutaneous peripheral T-cell lymphomas were classified following the updated Kiel classification. Of these, 20 cases belonged to the low-grade group (T-cell chronic lymphocytic leukaemia, 3; lymphoepithelioid, 5; angioimmunoblastic, 4; pleomorphic small cell, 8) and 21 to the high grade group (pleomorphic medium and large cell, 11; immunoblastic, 3; large-cell anaplastic Ki-1 positive, 7). Seventy per cent showed a CD4+/CD8-phenotype, 39% a defective phenotype and 88% an activation phenotype. Eighty per cent had B-symptoms, 63% hepatomegaly, 48% splenomegaly and 26% had involvement of more than three lymphoid areas. Bone marrow was infiltrated in 34%, central nervous system in 4%, lung in 12% and skin in 14.6%. Seventeen per cent presented with extranodal disease and 82.8% had stage III/IV disease. Hypergammaglobulinaemia was found in 29%, hypercalcaemia in 7%, raised LDH serum levels in 58% and HTLV-I antibodies in only one case. Of the 37 treated patients 18 (48%) achieved a complete remission, but 33% relapsed. Mortality was 59% and actuarial overall survival at 38 months was 0.32. In the comparison of the clinical, analytical and immunophenotypic variables and outcome between low and high grade groups, only the average of bone marrow infiltration in the low grade and stage I-II, presence of defective phenotypes and higher Ki-67 positivity in the high grade group were significantly different.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Peripheral T-cell lymphoma: a clinicopathological study of 41 cases and evaluation of the prognostic significance of the updated Kiel classification. 851 73

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

The clinical findings and prognosis in 15 patients with primary Ki-1 anaplastic large cell lymphoma (ALCL) were analyzed and compared with those of patients with T cell and B cell lymphoma and Hodgkin's disease. Clinical data revealed lymphadenopathy in 13 patients (87%) and skin lesions in eight (53%). Other organic involvements were hepatomegaly in two patients (13%), splenomegaly in five (33%), and bone marrow involvement in three (20%). The rate of skin involvement was significantly higher than that in B cell lymphoma and Hodgkin's disease. In laboratory findings the gamma-globulin concentration was significantly higher than that in T cell lymphoma, and the erythrocyte sedimentation rate (ESR) was significantly higher than that in B cell lymphoma. Complete remission was achieved in 11 patients (73%) and the five-year relapse-free survival was 27%. The overall survival was 4.0-69.8 months (mean 30.6 months). The mean survival was compatible with that of T cell lymphoma and was significantly shorter than that in Hodgkin's disease. Ki-1 ALCL can be distinguished from other lymphomas clinically as well as pathologically. Because Ki-1 ALCL is chemosensitive and the prognosis is as poor as that of T cell lymphoma, aggressive chemotherapy should be employed for the treatment of this disease.
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PMID:Clinicopathological characteristics of primary Ki-1 anaplastic large cell lymphoma. 871 76

The clinical histopathological and immunophenotypic features in 5 patients with Ki-1 positive non-Hodgkin's lymphoma (NHL) were studied. When first seen, 4 patients presented enlargement of superficial lymph nodes, with skin lesions in 2 patients. Two patients in stage IV with fever, hepato-splenomegaly and bone marrow invasion, died. Histologically, the tumor cells showed diffused or patchy hyperplasia. The cells were relatively large in size, rich in bosophilic or slightly eosinophilic cytoplasm with irregularly-shaped nuclei, prominent nucleoli, and distinct anaplasia and pleomorphism. Some of the cells looked very much like the Reed-Sternberg cells. Multinucleated giant cells were seen. Immunophenotypically, all the cells were CD30 (Ki-1) and CD25 (IL-2 receptor) positive but CD15 (Leu M1) negative. Thus, the 5 patient T Ki-1 positive NHL were all of T cell type.
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PMID:[Studies of the characteristic features of Ki-1 positive non-Hodgkin's lymphoma]. 938 28