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)

Immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.
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PMID:Immunoblastic lymphadenopathy with purpura and cryoglobulinemia. 30 Oct 9

Visceral leishmaniasis (VL) caused by Leishmania donovani, a protozoan parasite, is a disease of high morbidity associated with hepatosplenomegaly, hypergammaglobulinemia, fever and death. One of the immunological hallmarks of VL is a remarkable increase in serum immunoglobulin levels as a result of polyclonal B cell activation. This study demonstrated that T lymphocytes expressing the T cell receptors (TcR) gamma delta in association with CD3 molecules are increased in circulation of patients with VL. A large proportions of TcR gamma delta-bearing T cells had CD4+ CD8- phenotype, and expressed CD25, CD38, CD71 and HLA-DR activation antigens. Furthermore, we demonstrated wide functional differences in TcR gamma delta and TcR alpha beta T cells in their proliferative response, secretion of interleukin-2 (IL-2), B cell growth factor (BCGF) and B cell differentiation factor (BCDF). It was of interest that the TcR gamma delta T cells from patients with VL could be expanded by in vitro culture with human recombinant IL-2. Although these TcR gamma delta T cells secreted diminished levels of IL-2, they produced highly augmented levels of both BCGF and BCDF, suggesting that secretion of these lymphokines in these T cell subsets is regulated independently. The relative increases in the CD4+ CDw29+ TcR gamma delta T cell subsets and their secretion of highly elevated levels of BCGF and BCDF largely accounted for the humoral immune system abnormality and hypergammaglobulinemia found in this disease. These observations may help to explain that TcR gamma delta T cells might be functional in vivo and are involved in immunological mechanisms of pathogenesis in VL.
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PMID:Gamma delta T cells and the immune response in visceral leishmaniasis. 137 36

Localized plasma cell type Castleman's disease (CD) is an unusual pathologic entity. It is frequently associated with clinical and laboratory characteristics and rarely occurs in children. Total surgical excision results in cure in all aspects. To make early diagnosis of mesenteric CD is not easy, especially for children. An 11-year-old Taiwanese boy was recently evaluated for anemia and delayed growth. His clinical findings included a syndrome of severe hypochromic microcytic anemia, neutropenia, thrombocytosis, hypoferremia, hypergammaglobulinemia, and growth failure. Radiological examinations (abdominal ultrasound, small intestinal series, and computerized tomography) identified hepatosplenomegaly, nephromegaly, and huge masses in the middle abdomen with precaval, celiac, and paraaortic lymph nodal enlargement. However, detailed physical examination failed to detect a mass. At laparotomy a double-fist-sized confluent mass was found arising from the mesenteric root. Most masses were discrete and were excised individually. The pathologic diagnosis was plasma-cell type angiofollicular lymph node hyperplasia (Castleman's disease). Seven weeks after surgery, he had an episode of acute hepatitis B. Postoperatively, he exhibited a dramatic growth spurt; the hemoglobin, red blood cell indices, serum iron, and immunoglobulins returned to normal in 2 months. Neutropenia, which has not been previously related to mesenteric CD, was an unexpected finding in our case; however, it resolved spontaneously 3 months after the surgery, suggesting its causal relationship with the tumor.
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PMID:New observations in a child with angiofollicular lymph node hyperplasia (Castleman's disease) originated from the mesenteric root. 151 Jan 96

We report the case of 43-year-old homosexual patient with HIV infection and a history of travel to the Far East in whom visceral leishmaniasis was the first infectious complication. Symptoms were fever, malaise, weight loss, hepatosplenomegaly, generalized lymphadenopathy, and oral thrush. Laboratory abnormalities included a slight elevation of liver enzymes, impairment of liver function tests, leukocytopenia, anemia, hypergammaglobulinemia, and markedly depressed CD4(+)-cell counts. Despite initially successful treatment with pentavalent antimony, a relapse of leishmaniasis occurred after 7 months. Eradication of the infection was not achieved. Treatment was continued as a palliative chronic suppressive treatment with fortnightly pentamidine infusions. The clinical course was complicated by legionella pneumonia and the development of rapidly progressing Kaposi's sarcoma. The case is presented in detail, and the influence of HIV infection on the course of leishmaniasis is discussed.
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PMID:Visceral leishmaniasis in an HIV-infected patient: clinical features and response to treatment. 166 24

Angioimmunoblastic lymphadenopathy (AILD), first discovered in the 70's, is an infrequent disease which generally proves fatal within a short time. It is characterized by lymphadenopathies, hepatosplenomegaly, fever and rash. The most frequent laboratory findings are: anemia, leukocytosis with lymphopenia and non specific hypergammaglobulinemia. In spite of being considered a non malignant disease, it produces important immunity disorders which predispose the patient to serious infections, frequently fatal. In the course of time patients are likely to develop malignant lymphomas or other types of tumors. We describe a ganglionar proliferation and general symptoms in a patient who had been diagnosed as AILD by ganglionar biopsy. She was treated with corticosteroids during 8 months after which she had a complete recovery. Three months later the patient was readmitted with enterrorhagias and clear deterioration of her general condition. Tests showed the existence of a colon tumor and absence of adenomegaly in the areas previously affected by AILD. A colon surgery was carried out and a specimen examined. The anatomopathologic examination confirmed the existence of an immunoblastic lymphoma infiltrating the regional ganglionar area. Colon lymphomas constitute only 4% of all colon tumors; they are related to chronic gastrointestinal disease such as Crohn disease, ulcerative colitis, malabsorption syndromes, tumors and others. We conclude that in this patient AILD and prednisone administration constituted favoring factors for the development of an extranodal lymphoma.
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PMID:[Angioimmunoblastic lymphadenopathy and colonic lymphoma]. 213 Feb 46

Epstein-Barr virus (EBV) DNA was detected in polyclonal T cells that proliferated transiently in a 21-year-old male (referred to as H.J.) who underwent an apparently benign lymphocytosis (white blood cells, 31 x 10(6)/microL; lymphocyte, 79%) with fever, tonsillar swelling, lymphadenopathy, and hepatosplenomegaly. The symptoms and signs subsided mostly within a month of hospitalization. The major population of the lymphocytes at admission was positive for CD3, CD8 (4/8 ratio, 0.16), WT31, and DR antigen. Eight percent of the leukocytes were too blastoid to be classified as atypical lymphocytes of infectious mononucleosis (IM). The blastoid lymphocytes and the duration and degree of the lymphocytosis and hypergammaglobulinemia appeared inconsistent with IM, whereas the EBV serology indicated either EBV primary infection or a secondary alteration of normal seropositive EBV immunity. The genomic analysis of T-cell receptor beta chain in the peripheral blood mononuclear cells (PBMC) at admission with a C beta probe did not show a monoclonal rearrangement. EBV genome was detected in these cells, using the BamHI W and K probe, but not in the cells after discharge. Analysis of the EBV terminal repeat junctional sequence, using Xho I fragment of the latent membrane protein (LMP) probe binding with the terminus, did not show monoclonal or oligoclonal populations. EBV-associated nuclear antigen (EBNA) was detected in 36% of the PBMC at admission, but not in the later cells. These EBNA-positive cells were found to form rosette with sheep erythrocytes. The PBMC of six acute IM patients contained neither EBV DNA nor EBNA-positive cells. The observations in this case show a unique type of EBV infection in T cells that has not been previously reported.
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PMID:Detection of Epstein-Barr virus genome in benign polyclonal proliferative T cells of a young male patient. 216 93

To elucidate the clinicopathologic features of non-Hodgkin's lymphoma (NHL) in Taiwan, 123 adult patients with proven NHL were studied. They were classified according to the international working formulation as: low grade (LG), 12.2%; intermediate grade (IG), 42.3%; and high grade (HG) lymphoma, 45.5%. The most common subtypes were diffuse large cell (26.8%) and large cell immunoblastic (26.8%) lymphomas. Follicular lymphoma accounted for only 8.9% (11 cases). Complete remission rates for LG, IG and HG lymphomas were 53%, 35% and 34%, respectively. LG lymphoma had a significantly better survival than that of IG and HG lymphomas. The IG lymphoma encompassed a heterogeneous group of patients with varying prognoses but the overall survival curve was indistinguishable from that of HG lymphoma. Clinically, 66% of HG, 77% of IG and 86% of LG lymphoma presented with advanced disease. LG lymphoma had high frequencies of hepatosplenomegaly (30-50%) and bone marrow involvement (53%), whereas skin, bone and central nervous system involvement occurred exclusively in IG and HG lymphomas. Mild anemia was common and occurred in 40-50% of the patients. Hyperimmunoglobulinemia was found in 50-60% of all 3 grades of lymphoma, monoclonal gammopathy in 3 cases of IG lymphoma, and hypercalcemia in 4 cases of IG and HG lymphomas. Elevated serum lactate dehydrogenase occurred mainly in IG and HG lymphomas and was an important prognostic factor. In conclusion, the characteristic features of NHL in Taiwan include: (1) a high proportion of HG lymphoma and low proportions of LG and follicular lymphomas; (2) a heterogeneous patient composition of IG lymphoma with an unfavorable overall prognosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Adult non-Hodgkin's lymphoma in Taiwan area: a clinicopathologic study of 123 cases based on working formulation classification. 276 10

An eight-year-old child from Zaire died in Sweden in 1982 after a clinical course compatible with the acquired immunodeficiency syndrome (AIDS). In 1975, at the age of 5 months, the infant had an acute viral infection with a rash; this illness was followed by a chronic cough. During the course of the disease he had recurrent septicemia, fever (frequently with miliary lung infiltrates), disseminated lymphadenopathy, hepatosplenomegaly, candidiasis, and diarrhea. Late in the illness the child developed lethal disseminated disturbances of the central nervous system. Immunologic investigations revealed a pronounced hypergammaglobulinemia, normal C3 but low C4 values, decreased number of T-lymphocytes, and decreased lymphocyte stimulation with T-cell and B-cell mitogens. Samples of serum taken in 1981 and 1982 were analyzed and found to be positive for antibodies to HTLV-III virus. The course of the disease in this child was more prolonged than most of the pediatric cases described earlier. It is likely that this child developed AIDS early in 1975, long before the AIDS epidemic was apparent in the United States.
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PMID:Early case of acquired immunodeficiency syndrome in a child from Zaire. 301 6

The clinical and immunologic abnormalities of 7 infants with Lymphadenopathy Associated Virus (LAV) infection are reported. Indicative immune changes, positive anti-LAV serologic test and/or virus isolation led to the diagnosis. In a case with a particularly severe form of the disease, whose mother died from AIDS, serologic tests and virus isolation were negative. Beside a case associated with blood transfusion, the familial and ethnic context helped the diagnosis. Clinical and biochemical features were close to those in adult AIDS, including hepatosplenomegaly, polyadenopathy, a decreased absolute number of OKT4(+) lymphocytes and hypergammaglobulinemia. The importance of cellular and sometimes also humoral immune deficiency was highly variable. For 4 patients, the severity of the immune deficiency allowed the diagnosis of AIDS. Occurrence of such an infection in the first months of life suggests a fetomaternal viral transmission; however, a postnatal contamination cannot be ruled out. Long-term prognosis is difficult to assess. It depends, among other factors, on the importance of the immune deficiency.
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PMID:[LAV infections and the acquired immunodeficiency syndrome in infants]. 301 18

An infant of Arab extraction with the Type II form of Gaucher's disease is described. His clinical presentation was unusual because in addition to the extensive neurological involvement and marked hepatosplenomegaly a severe congestive cardiomyopathy and renal tubular dysfunction were present. In addition, marked hypergammaglobulinemia and raised serum angiotensin converting enzyme levels were found. It is suggested that these varied manifestations may be ascribed to the consequences of glucocerebroside deposition within the macrophages of the reticuloendothelial system.
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PMID:The varied clinical and laboratory manifestations of type II Gaucher's disease. 303 64


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