Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

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

CD30/Ki-1 positive anaplastic large cell lymphoma (Ki-1 ALCL) frequently exhibits extranodal disease and chromosomal t(2;5)(p23;q35). An 11-year-old girl presented with an intramuscular tumor of the right upper arm. Tumors of the chest wall, left arm and leg, hepatomegaly, pleural effusion, and enlarged lymph nodes then developed. The intramuscular tumor and pleural effusion showed a diffuse infiltration of large atypical cells with abundant amphophilic cytoplasms. The tumor cells were positive for CD30, CD2, CD45RO, and p80, but were negative for other T-cell, B-cell, and myeloid cell antigens. She was diagnosed as having Ki-1 ALCL with a T-cell origin. Cytogenetic studies showed an abnormal karyotype including a der(17)t(1;17)(q11;p11). She received seven cycles of intensive chemotherapy followed by an autologous peripheral blood stem cell transplantation, and has been in complete remission for more than two years. The primary involvement of skeletal muscle is quite uncommon in ALCL, and an abnormal karyotype including t(1;17)(q11;p11) has not been reported previously. Since a high frequency of aberrations of 1p36/1q12 or 17p13.3 was detected in sarcoma cells, the presence of suppressor genes is suggestive in these sites.
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PMID:Primary CD30/Ki-1 positive anaplastic large cell lymphoma of skeletal muscle with der(17)t(1;17)(q11;p11). 1110 21