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)

Kikuchi's disease is characterized by lymphadenopathy in young patients and may be mistaken for malignant disease both clinically and histologically. We report the case of a 26-year-old man with persistent fever for 3 weeks and splenomegaly, in whom pancytopenia developed afterwards. A bone marrow aspiration was normal. Blood, urine, throat, stool and bone marrow cultures were negative as were serological tests for lues, Toxoplasma, Epstein-Barr virus and Widal's test. An abdominal CT scan showed enlarged retroperitoneal lymph nodes and an exploratory laparotomy was performed. Two lymph nodes were excised and a wedge biopsy of the liver was performed. The histological findings in the lymph nodes were compatible with the diagnosis of Kikuchi's disease. The patient became afebrile on the 2nd postoperative day without any treatment. He has been well for 4 months after discharge.
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PMID:Kikuchi's necrotizing lymphadenitis: a cause of fever of unknown origin and splenomegaly. 312 82

A detailed clinicopathologic analysis of 30 patients with sporadic fatal infectious mononucleosis and 31 males with fatal infectious mononucleosis and the X-linked lymphoproliferative syndrome was performed to determine the extent of hepatic dysfunction in these cases. At death, the median age of patients with sporadic infectious mononucleosis was 10.7 yr vs. 2.4 yr for X-linked lymphoproliferative syndrome. The median survival time was 8 wk for sporadic infectious mononucleosis and only 4 wk for X-linked lymphoproliferative syndrome. The male to female ratio was 3:2 in sporadic infectious mononucleosis; all patients with X-linked lymphoproliferative syndrome were males. Fever, sore throat, lymphadenopathy, hepatomegaly, and splenomegaly were prominent findings. Hepatic dysfunction was uniformly present and caused death in 13 of 30 sporadic infectious mononucleosis cases and 18 of 31 X-linked lymphoproliferative syndrome cases. Diagnosis of infectious mononucleosis was confirmed by heterophile antibody titers or Monospot, Epstein-Barr virus antibody studies, viral culture, molecular hybridization studies, clinical and histologic findings, and pedigree analysis.
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PMID:Hepatitis in fatal infectious mononucleosis. 367 38

Certain lymphoproliferative disorders (Burkitt's lymphoma, tropical splenomegaly, and Mediterranean lymphoma) are more frequently observed in the tropical or subtropical areas than in other parts of the world. The polymicrobial exposure of human beings in those areas to viral, bacterial and protozoal infections led to the assumption of a specific connection between these conditions and the lymphoproliferative disease. Some evidence has accumulated that the Epstein-Barr virus might be an oncogenic virus connected with the development of Burkitt's lymphoma. Repeated plasmodial infections were more tenuously thought to be associated with tropical splenomegaly and, similarly, Giardia lamblia or Vibrio cholerae infections with immunoproliferative small-intestinal disease. However, those connections are only speculative and by no means proven. Small-intestinal lymphoma represents an example of a wide spectrum of manifestations covering autonomous pre-malignant immunoproliferative stages, als well as the full-blown malignant stages. Polyclonal activation and proliferation of B-lymphocytes, as well as concomitant immunosuppression are the characteristic features of the pre-malignant situation and may be the basis for the malignant transformation of single cells. Thus, the final clinical picture resembles that of a malignant non-Hodgkin lymphoma.
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PMID:[Lymphatic diseases specific to the tropics (Burkitt's lymphoma, tropical splenomegaly and Mediterranean lymphoma)]. 608 44

A new wild-type isolate of Epstein-Barr virus (EBV) was identified in follow-up studies of a case of chronic active EBV infection in an 8-year-old girl who had high titres of antibody to viral capsid antigen and early antigen (EA) (greater than 20 480 and 2560 respectively), persistent splenomegaly and abnormal immunologic features. More than 10 throat washings from this patient failed to transform cord blood lymphocytes (CBL), but at least 7 were able to induce EA in Raji cells. Supernatants from cultures of the lymphoblastoid cell line obtained by in-vitro infection of this patient's leukocytes with the B95-8 strain of EBV revealed a herpesvirus particle when examined by electron microscopy. The same supernatants were unable to transform CBL but could induce EA in Raji cells upon superinfection. In 30 or more trials the patient's lymphocytes never transformed spontaneously but did become positive for EBV nuclear antigen and EA in the first week of culture at least twice. Parallel studies performed on the father of the patient yielded similar results. This, then, is the first report documenting lytic activity associated with a wild-type EBV isolate.
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PMID:Lytic, nontransforming Epstein-Barr virus (EBV) from a patient with chronic active EBV infection. 609 76

A complete blood count performed during the evaluation of a febrile or traumatized child may show the presence of atypical lymphocytosis. Although atypical lymphocytes occur in several conditions, they are most closely associated with infectious mononucleosis (IM). We initiated a study to determine if the presence in children of atypical lymphocytosis without the triad of splenomegaly, pharyngitis, and adenopathy is a manifestation of IM. Heterophil antibody (HA) and Epstein-Barr virus (EBV) titers were performed on 45 children with greater than or equal to 5% atypical lymphocytes. None had a significant HA titer. Thirty-three were seronegative for EBV, and 11 had titers indicative of past infection. One child had an antibody pattern suggesting a recent EBV infection; none had a titer consistent with acute IM. We conclude that isolated atypical lymphocytes does not point to infectious mononucleosis.
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PMID:Atypical lymphocytosis in children. 626 86

In two members of a family (daughter and father) active Epstein-Barr virus (EBV) infections persisted over periods of 4 and 3 years respectively (possibly 10 years in the father). Both had persistent splenomegaly and occasional bouts of unexplained fever but lived otherwise normal lives. The other members of the family (mother and son) were healthy. The titres of antibody to the EBV viral capsid antigen (VCA) and early antigen (EA) were extremely high in the daughter's blood, whereas the titres of antibody to the Epstein-Barr nuclear antigen were low in the daughter's blood and undetectable in the father's. Target cells of the EBV infection that were obtained from the daughter's blood were established in culture with great difficulty and showed increased expression of VCA and EA. Other immunologic investigations in the two patients revealed that the ratio of helper to suppressor T lymphocytes was inverted, natural killer-cell activity was abnormally low, lymphocyte responses to certain mitogens were depressed and there was a serum factor blocking mitogen-induced transformation. The possibility that the patients' unusual susceptibility to EBV infection represented an inherited syndrome (perhaps X-linked) is discussed.
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PMID:A familial syndrome of susceptibility to chronic active Epstein-Barr virus infection. 631 44

Although almost always a benign, self-limiting disease, infectious mononucleosis accounts for considerable symptomatic illness in the young athlete and can, on occasion, be truly life-threatening. Recognition of the syndrome "glandular fever," vis-a-vis infectious mononucleosis--fever, pharyngitis, lymphadenopathy, and splenomegaly, with characteristic changes in the peripheral blood leukocytes--dates back over a half a century. However, seroepidemiologic studies have only recently established its viral causation and epidemiology. This acute infection by the Epstein-Barr virus is unique pathophysiologically--an acute, self-limiting, lymphoproliferative disorder with autoimmune features--and may well be the cause or one of the causes of several malignant neoplasms, Burkitt's lymphoma, and nasopharyngeal carcinoma. This review (1) describes infectious mononucleosis, pathophysiologically, clinically, and epidemiologically; and outlines its most frequent and serious complications; (2) discusses how to reliably diagnose infectious mononucleosis and evaluate the heterophile-negative case; and (3) addresses management, especially the thorny issues of the use of corticosteroids and restriction from athletic training and participation.
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PMID:Infectious mononucleosis in the athlete. Diagnosis, complications, and management. 711 52

Clonal expansions of CD3+ large granular lymphocytes (LGL) have been classified as T-LGL leukemia. The majority of patients with T-LGL leukemia have a chronic disease (years) manifested often by severe neutropenia, rheumatoid arthritis, and mild-to-moderate splenomegaly. The characteristic phenotype of the leukemic LGL is CD3+, CD8+, CD16+, CD57+, and CD56-. In this report we describe an aggressive variant of T-LGL leukemia in which leukemic LGL also expressed CD56, as identified by two-color flow-cytometry analysis. In contrast to the chronic nature typical of T-LGL leukemia, these patients presented with a severe systemic illness that was rapidly progressive and resistant to treatment. Atypical clinical features included rapidly increasing spleen size to massive proportions, extensive lymphadenopathy, and the presence of B symptoms (fever, nightsweats, weight loss). Hematologic and pathologic features were also unusual for T-LGL leukemia. These patients had very high LGL counts at diagnosis (range 11,692 to 26,312 microL), which increased rapidly despite treatment. Histopathologic examination of splenic sections showed extensive infiltration of red pulp cords and sinuses by leukemic cells with atrophy of the white pulp. These clinicopathologic features are similar to those described for patients with natural killer cell (NK)-LGL leukemia, whose cells are also CD56+. However, unlike NK-LGL leukemia, we could not show a direct pathogenic role for Epstein-Barr virus (EBV), as Southern-blot analyses using an EBV-joined termini probe were negative in these patients. Our findings suggest that CD3+, CD56+ LGL leukemia is a distinct clinicopathologic entity separate from the usual CD3+, CD56- T-LGL leukemia. The expression on leukemic LGL of CD56, an adhesion molecule, may determine the aggressive biologic nature of this newly described disease.
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PMID:CD3+, CD56+ aggressive variant of large granular lymphocyte leukemia. 754 72

A two year old boy exhibited not only clinical manifestations which suggested a recurrence of Kawasaki disease (KD) but also evidence of a primary infection by Epstein-Barr virus (EBV) including tonsillitis, splenomegaly and atypical lymphocytosis in the peripheral blood. An inverted CD4/CD8 ratio in lymphocyte subsets suggested the presence of infectious mononucleosis (IM). Epstein-Barr virus titers (viral capsid antigen-immunoglobulin G 1:20; Epstein-Barr virus-associated nuclear antigen < 1:10) showed an acute EBV infection and the presence of EBV genome in the blood was determined by the polymerase chain reaction technique. In Japan, the peak incidence of KD and IM is in children under 4 years of age. From the investigation of EBV titers, it has been reported that some patients with KD develop an associated, unusual primary EBV infection. Kawasaki disease concurrent with a primary EBV infection as in this case, suggests the possibility of an etiologic agent related to the KD rather than to the EBV infection itself.
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PMID:Kawasaki disease with a concomitant primary Epstein-Barr virus infection. 787 90

Acute idiopathic thrombocytopenic purpura (ITP) often appears to be related to the sensitization by some viral infections. However, the causative viral agents are not identified in most cases. Although the primary infection with Epstein-Barr virus (EBV) occurs during early childhood in Japan, the majority of cases are usually asymptomatic. A minority are associated with acute infectious mononucleosis (IM), which is characterized by fever, tonsillitis, lymphadenopathy, splenomegaly and liver dysfunction. In this report, three cases are described of children with EBV-induced ITP who clinically had atypical findings of IM. Their primary EBV infections were confirmed by serological test and, in addition, were verified by the enhanced expression of activation antigens (HLA-DR and CD45RO) on T cells as well as the inverted ratio of CD4+ to CD8+ subsets. These observations imply that ITP can occur as one of the host responses during primary EBV infections, irrespective of clinical manifestations. Evaluation of lymphocyte subpopulations may be useful for the assessment of primary EBV infection in ITP.
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PMID:Acute thrombocytopenic purpura associated with primary Epstein-Barr virus infection. 794 9


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