Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

---

Query: UMLS:C0019214 (hepatosplenomegaly)
4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic active Epstein-Barr virus (CAEBV) infection syndrome is a heterogeneous EBV-related disorder characterized by chronic fatigue, fever, lymphadenopathy, and/or hepatosplenomegaly, associated with abnormal patterns of antibody to EBV. CAEBV can range from disabling mild/moderate forms to rapidly lethal disorders. Even patients with mild/moderate disease frequently suffer adverse effects from long-term anti-inflammatory agents and have a quality of life that progressively deteriorates. It is still unknown why these individuals are unable to produce an effective immune response to control EBV, and no effective treatment is currently available. Since ex vivo-expanded EBV-specific cytotoxic T lymphocytes (EBV-CTLs) can safely restore EBV-specific cellular immune responses in immunodeficient patients, we assessed the possibility that adoptive immunotherapy might also effectively treat CAEBV infection. Following stimulation with irradiated EBV-transformed lymphoblastoid cell lines (LCLs), EBV-CTLs were successfully generated from 8 of 8 patients with the mild/moderate form of CAEBV infection. These CTLs were predominantly CD3(+) CD8(+) cells and produced specific killing of the autologous LCLs. There were 5 patients with 1- to 12-year histories of disease who were treated with 1 to 4 injections of EBV-CTLs. Following infusion, there was resolution of fatigue and malaise, disappearance of fever, and regression of lymphadenopathy and splenomegaly. The pattern and titers of anti-EBV antibodies also normalized. No toxicity was observed. There were 4 patients who did not show any relapse of disease within 6 to 36 months follow-up; one patient had recurrence of fatigue and myalgia one year after CTL infusion. We suggest that adoptive immunotherapy with autologous EBV-CTLs may represent a safe and feasible alternative treatment for patients affected with mild/moderate CAEBV infection and that this approach should be evaluated in the more severe forms of the disease.
...
PMID:Autologous Epstein-Barr virus (EBV)-specific cytotoxic T cells for the treatment of persistent active EBV infection. 1239 55

We describe a case of B-prolymphocytic leukaemia (B-PLL) who, following a long-lasting remission with fluradabine, developed a Hodgkin's lymphoma (HL) with bone marrow involvement. A 75-yr-old male was found to have a lymphocytosis [white blood cell (WBC) count = 146 x 10(9) L(-1)], small volume axillary lymphadenopathy, and hepatosplenomegaly. The majority of circulating lymphocytes had a round nucleus and prominent single nucleolus. The patient did not respond to chlorambucil and then received fludarabine, achieving a good response lasting for 5 yr, when he manifested with B symptoms and pancytopenia. A diagnosis of HL in the bone marrow was made based on histology (Reed-Sternberg cells) and immunohistochemistry (CD30+, CD15+). Epstein-Barr virus (EBV) studies were negative. The patient was treated with chemotherapy but died 6 months later from disease progression.
...
PMID:Prolymphocytic leukaemia and Hodgkin's lymphoma. 1240 13

We report a rare case of composite angioimmunoblastic T-cell lymphoma (AILT) and diffuse large B-cell lymphoma occurring in a 48-year-old woman with generalized lymphadenopathy and hepatosplenomegaly. The patient initially sought care at a local hospital with a single enlarged left cervical lymph node. Histologic examination of the node was interpreted as an atypical immunoblastic proliferation. She developed generalized lymphadenopathy 10 months later and was referred to our institution for further evaluation. The recent biopsy of the cervical node showed typical features of AILT Flow cytometric immunophenotyping identified an aberrant CD4+ T-cell population that lacked surface CD3. Polymerase chain reaction analysis of the T-cell receptor gamma gene revealed a clonal rearrangement. In addition to the AILT, the lymph node showed partial involvement by a diffuse large B-cell lymphoma. The B lymphoma cells and admixed immnunoblasts and Reed-Sternberg-like B cells in the AILT were positive for Epstein-Barr virus (EBV) by in situ hybridization. Ourfindings raise the possibility that the EBV-associated large B-cell lymphoma is a secondary event in AILT via EBV infection or reactivation followed by clonal expansion of an immortalized EBV-infected B cell clone.
...
PMID:Composite angioimmunoblastic T-cell lymphoma and diffuse large B-cell lymphoma: a case report and review of the literature. 1247 77

An 11-year-old boy had intermittent fever and hepatosplenomegaly. The diagnosis of chronic active Epstein-Barr virus (EBV) infection was established from an abnormal pattern of anti-EBV antibody; EBV was detected in bone marrow cells. Immunochemotherapy alleviated the patient's symptoms. However, when a subsequent oral prednisolone dose was tapered, heart failure ensued and he died. Autopsy findings demonstrated that myocarditis resulted from infiltrating T lymphocytes with the EBV genome and a benign histologic appearance. A clonality study of T lymphocytes indicated no such evidence of monoclonality. EBV-infected T cells play an important role in the pathogenesis of myocarditis in chronic active EBV infection.
...
PMID:Polyclonal proliferation of lymphocytes containing the epstein-barr virus genome in a patient dying of myocarditis in chronic active Epstein-Barr virus infection. 1254 81

We report a case of aggressive natural killer (NK) cell lymphoma/leukemia in a 70-year-old woman presenting with fever, hepatosplenomegaly, retroperitoneal lymphadenopathy, and elevated serum CA19-9. The patient died 4 days after diagnosis. Neoplastic NK cells were identified in the blood and retroperitoneal lymph node biopsy. Their phenotypes were confirmed by extensive flow cytometric and immunohistochemical studies. In situ hybridization for Epstein-Barr virus (EBV)-associated RNA (EBER) was positive. Various forms of NK cell neoplasm were reviewed and discussed.
...
PMID:Aggressive natural killer cell lymphoma/leukemia. 1263 10

A 66-year-old man was admitted to our hospital for fever on January 19, 1998. He began showing periodic high fever in June 1997 and an increased serum LDH in August 1997. His history included surgery for esophageal cancer in 1993. On admission, the patient's body temperature was 38.5 degrees C. Physical examination was negative for lymphadenopathy, hepatosplenomegaly, and skin rash. Peripheral blood revealed a hemoglobin level of 8.6 g/dl and a platelet count of 7.9 x 10(4)/microliter. Bone marrow examination showed hypocellularity with marked histiocytic hemophagocytosis. The various bacterial cultures were negative. Serum LDH was elevated to 1,606 IU/l, and ferritin was greater than 3,000 ng/ml. Antinuclear antibodies were negative. No significant elevation of viral antibody titers including that to Epstein-Barr virus was found. Hemophagocytic syndrome (HPS) was diagnosed, but no underlying diseases was identified. The patient's condition was complicated by interstitial pneumonia and pleural effusion. gamma-globulin and pulse methylprednisolone both proved ineffective for the HPS; however, complete remission was achieved with cyclic intravenous administration of etoposide (VP-16, 150 mg/day). Interestingly, the interstitial pneumonia resolved promptly with etoposide therapy. The patient relapsed, in July 2001, exhibiting high fever, cytopenia, and marrow hemophagocytosis. His condition was ameliorated by administration of etoposide. This was a rare case of chronic and recurrent HPS of unknown etiology accompanied by interstitial pneumonia. Etoposide should be considered as a primary therapy for HPS and its complications in cases such as our patients.
...
PMID:[Successful use of etoposide in an elderly patient with chronic recurrent hemophagocytic syndrome]. 1270 51

We report a case of chronic active Epstein-Barr (EB) virus infection (CAEBV) associated with skin eruptions mimicking hydroa vacciniforme (HV) in a 4-year-old boy. The patient had repeated episodes of vesiculo-necrotic eruptions on the face, scalp, and bilateral forearms one year before the first visit to our department. General symptoms including fever, hepatosplenomegaly, abnormal liver function, and cervical lymph node swelling were noted three months before the first visit. At the first visit, small, bean-sized, erythemic papules with central necrosis were observed on the face and anterior chest wall. Thumb-sized ulcers with crust were present on the bilateral forearms. Histopathological examination of an erythematous lesion in the submandibular area revealed parakeratosis with a thick crust, mild spongiosis in the epidermis, and a dense infiltration of lymphoid cells into the dermis and perivascular space. Laboratory examination showed EBNA x 40, EBV VCA IgG x 1,280, and EBV DNA (PCR) 8 x 10(4). EBV-encoded small nuclear RNA (EBER) positive cells were detected in the dermis by an in situ hybridization (ISH) method. Large granular lymphocytes (65%) with the NK cell phenotype were found in the peripheral blood. A real time PCR method showed 171,741 copies/ micro g DNA in CD 16 positive cells. Although latent EBV infection-associated eruptions have been documented, detailed skin manifestations in CAEBV are less well known.
...
PMID:Hydroa vacciniforme-like eruptions in a patient with chronic active EB virus infection. 1277 6

We report here a case of mantle cell lymphoma (MCL) in a patient who, following Epstein-Barr virus (EBV) infection, developed diffuse large B-cell lymphoma (DLBCL). A 47-year-old woman was diagnosed as having MCL with clinical stage IIIA in July 1990. After treatment with a third-generation chemotherapy without response, she was kept under observation for 8 years. In January 1999, fever and night sweats appeared with laboratory evidence for EBV infection, and acute swelling of lymph nodes and hepatosplenomegaly developed in May 1999. Histopathological examination confirmed the diagnosis of DLBCL. Sequence analysis of the complementarity-determining region (CDR)-III of the immunoglobulin heavy chain gene demonstrated clonal identity between the initial MCL and the subsequent DLBCL. Immunohistochemistry revealed that cyclin D1, CD5, and CD20 were expressed in the MCL but lost in the DLBCL cells, and EBER-ISH confirmed that EBV infection was absent in the former but present in the latter. Southern hybridization with the EBV terminal repeat probe showed a clear monoclonal pattern in the DLBCL specimen. All these results suggest that EBV infection may have been the molecular event that caused transformation of MCL cell(s) to DLBCL in this case. This is, to the best of our knowledge, the first well-documented case of EBV-associated transformation of MCL.
...
PMID:Case of Epstein-Barr virus-associated transformation of mantle cell lymphoma. 1282 58

Hepatosplenic T cell lymphoma is defined as an extranodal and systemic neoplasm derived from cytotoxic T cells. This report describes a postmortem case of T cell lymphoma that showed histological features of hepatosplenic T cell lymphoma but did not express cytotoxic molecules. The patient was a 57 year old man who presented with severe icterus and hepatosplenomegaly, followed by an aggressive clinical course. The liver and spleen were enlarged, weighing 2000 g and 360 g, respectively. Histologically, the liver, spleen, and bone marrow were entirely affected by lymphoma, comprising pleomorphic small and large cells, which displayed sinusoidal infiltration in the liver, diffuse infiltration in the splenic cord, and interstitial/diffuse infiltration with fibrosis in the bone marrow. Lymphoma cells showed positivity for CD3 epsilon, CD8, and CD45RO and clonal rearrangement of the TCRgamma gene by the polymerase chain reaction on paraffin wax embedded sections. However, they were negative for TIA-1 and granzyme B, in addition to betaF1, CD4, and CD56. Few neoplastic cells were stained for Epstein-Barr virus encoded mRNA 1. These findings indicate that this case might represent a variant of hepatosplenic T cell lymphoma despite the absence of cytotoxic molecules.
...
PMID:Hepatosplenic T cell lymphoma with no expression of cytotoxic molecules. 1289 Aug 21

Infectious mononucleosis (IM) is the manifestation of primary infection with Epstein-Barr virus (EBV). EBV persisting after infection for a life-time infects > 90% of the adult population. Primary infection mostly asymptomatic in young children manifests in teenagers and young adults in about 50% as IM with fever, sore throat, generalized lymphadenopathy, frequently hepatosplenomegaly and blood lymphocytosis with the characteristic atypical lymphocytes. Clinical presentation, typical lymphocytosis and heterophile antibodies are diagnostic. Atypical cases may need to be confirmed by specific serology. IM is a self-limiting lymphoproliferation regressing within 2-3 weeks. Complications are rare and may involve many different organs. Severe cases are very uncommon, except in patients with inborn or acquired immunodeficiency carrying a substantially higher risk for severe courses, pogredient lymphoproliferation and lymphoma.
...
PMID:[Infectious mononucleosis]. 1461 Sep 1


<< Previous 1 2 3 4 5 6 7 8 9 10

---