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)

It has been recently demonstrated that the Epstein-Barr virus (EBV) can infect human thymocytes and may be involved in the T cell neoplasms, in addition to African Burkitt's lymphoma, nasopharyngeal carcinoma and Hodgkin's disease. Four distinct clinicopathologic categories of EBV-associated T cell malignancies have been recognized. The angiocentric T cell lymphoma or lymphomatoid granulomatosis involving the nose (or midline lethal granuloma) and skin is frequently EBV-associated. The other 3 groups include angioimmunoblastic lymphadenopathy-like lymphoma, node-based T immunoblastic lymphoma which may contain Reed-Sternberg-like giant cells (Hodgkin's-like lymphoma), and T cell lymphoma resembling malignant histiocytosis. Both the CD4 and CD8 T cell subsets, and a hitherto undefined T lineage lacking CD4/CD8 expression have been involved. The common clinical features are prolonged fever, skin lesions, lymphadenopathy, hepatosplenomegaly, and pancytopenia. Serologic assays suggest that a chronic active EBV infection may exist in most of these patients. The EBV genomes appear to proliferate in clonal and episomal form in the neoplastic cells which show expression of latent membrane proteins. Although an indolent local phase may exist, the clinical course is aggressive for most patients with frequent development of drug resistance to conventional chemotherapy. EBV-associated T cell lymphoma constitutes a separate entity of virus-associated human diseases and opens a potential field to investigate the pathogenesis of EBV-associated human malignancies.
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PMID:Clinicopathological spectrum of Epstein-Barr virus-associated T cell malignancies. 133 23

The Epstein-Barr virus (EBV) has been shown to be associated with posttransplant lymphoma, Hodgkin's disease, and T-cell lymphoma, in addition to African Burkitt's lymphoma. In a retrospective study of 56 consecutive cases of T-cell lymphoma, EBV DNA was found by Southern blot and in situ DNA hybridization in 10 (20%) of 50 peripheral T-cell lymphomas, but in none of six cases of T-lymphoblastic lymphoma. Peripheral T-cell lymphomas containing EBV DNA could be subclassified into three categories according to histology and immunophenotypic studies: (1) T-cell lymphoma of the helper phenotype, five cases. Two cases had histologic features resembling angioimmunoblastic lymphadenopathy (AILD). (2) T-cell lymphoma of the cytotoxic/suppressor phenotype, four cases. AILD-like features could also be recognized in two cases. Reed-Sternberg-like giant cells were identified in three cases designated Hodgkin-like T-cell lymphoma. (3) Angiocentric T-cell lymphoma or lymphomatoid granulomatosis in one case, initially affecting the skin and nose; no T-cell subset could be defined. Six of the eight EBV DNA-positive patients tested for serum EBV antibodies had elevated titers of IgG antiviral capsid antigen (greater than 640) and/or early antigen (greater than 10). From combined studies of Southern blot hybridization by using EBV termini fragment probe and in situ DNA hybridization, the EBV genomes appeared to be clonotypically proliferated in the neoplastic T cells. The patients in all three groups usually had prolonged fever preceding the diagnosis, hepatosplenomegaly, an aggressive clinical course, and poor response to chemotherapy; nine died with a median survival of only 8 months. We propose that these EBV-associated aggressive T-cell lymphomas, like human T-cell leukemia/lymphoma virus-positive T-cell lymphoma, have characteristic clinicopathologic features and should be treated as a separate disease entity.
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PMID:Aggressive peripheral T-cell lymphomas containing Epstein-Barr viral DNA: a clinicopathologic and molecular analysis. 184 84

40 children (23 males, 17 females) have been diagnosed have ANLL during the period from february 1970 to september 1981. According to FAB classification, 24 cases were M1,-M2, 9 M3, 3 M4, 3 M5 and 1 M6. At diagnosis, 20 patients (50%) had leukocytes less than 10.000/mmc, 6 (15%) had leukocytes greater than 50.000mmc. Hb levels was 7 g% in 16 patients (40%); 10 children had hepatosplenomegaly (25%), 7 splenomegaly (18%) and 5 lymphoadenomegaly (13%). 4 patients had cutaneous or mucous infiltrates. None had meningeal involvement at diagnosis. According to the year of diagnosis, 3 groups can be identified. In the group I (1970-73), 11 patients have been treated with not codified combination chemotherapy as ARA-C, 6-TG, DNR, CTX, Metil-GAG. In the group II (1974-76) and in the group III (1977-81), the patients (respectively 12 and 17) have been treated according to the following protocols: LAM-5 (3), TRAP (5), COAP (1), LAM 80 (2), AIL 7402 (8), AIL 7604, AIL 7801 (6). Immunotherapy has been performed in 7 cases. CNS prophylaxis (MTX i.t. +/-ARA-C +/- RT) was given in 5 patients of group II and in 6 of group III. I patients of group I (45%), 6 of group II (50%) and 13 of group III (76%) achieved CR. Median duration of remission was 5 months in the group I and in 17 in group II and III.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Acute non-lymphatic leukemia in children]. 654 20

A 28-year-old man was admitted to our hospital, because of double vision, memory disturbance and dysarthria. These symptoms developed in November, 1994. His mental activity was gradually decreased and he became apathetic. A physical examination on admission was unremarkable. There were no lymphadenopathy, hepatosplenomegaly or skin rash. Neurological examination revealed disorientation, decreased mental activity and left gaze-evoked nystagmus. Neuropsychological tests demonstrated impairment of recent memory and performance but frontal lobe functions were maintained. Laboratory data showed hyponatremia, elevation of antinuclear antibodies and IgG-antibodies to Epstein-Barr virus capsid antigen and Epstein-Barr virus nuclear antigen. A brain MRI revealed multiple lesions in the cerebral subcortex, cerebellum and brainstem. These lesions showed high signal intensity on T2-weighted images and enhanced high signal intensity on T1-weighted images using Gd-DTPA injection. A cystic mass was seen between bilateral lateral ventricles and contacted with the fornix and thalamus. Lymphomatoid granulomatosis was diagnosed by an open brain biopsy, which showed diffuse infiltration of small lymphocytes, histiocytes and plasma cells, especially around the small vessels. Epithelioid granuloma or necrosis was not seen. An immunohistochemical study showed that nearly all lymphocytes were MT-1 positive T lymphocytes. The patient was treated by whole brain irradiation (30Gy) and pulse therapy of methylprednisolone. Multiple lesions in the brain responded to these modalities with complete resolution of the lesions and clinical improvement. In this case, impressive multiple and cystic lesions seen on brain MRI disappeared by whole brain irradiation and steroid. Since etiologies of multiple lesions on MRI includes many possibilities, histological diagnostic methods should be performed to obtain a definitive diagnosis. This is the first case of lymphomatoid granulomatosis that showed multiple and cystic lesions on brain MRI in Japan.
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PMID:[Lymphomatoid granulomatosis of the brain with multiple lesions on MRI]. 893 97

Lymphomatoid granulomatosis is an angiocentric lymphoreticular proliferative disease. Some patients go on to develop frank neoplasia. The authors describe a patient who presented at the age of 12 years with pulmonary nodules and hepatosplenomegaly and was followed up for 6 years, and who subsequently developed lymphoma after cessation of therapy.
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PMID:Lymphomatoid granulomatosis in a boy with long-term follow-up. 1155 32

A 45-year-old woman was admitted with complaints of non-productive cough, chest pain, fatigue and weight loss in the last 4 months. On physical examination moderate hepatosplenomegaly and crackles most notably on the basal region of the right lung were evident. Serial chest X-rays and computed tomographies revealed a migratory nodular infiltration pattern, changing in location and size in both the lungs. The histopathological diagnosis of the open lung biopsy was lymphomatoid granulomatosis (LG) with a marked angioinvasive lymphocytic perivascular and peribronchial infiltration pattern. In the immunohistochemical analyses LCA, CD-79, CD-20 were positive, while CD-30 was negative. No response could have been achieved under combination chemotherapy and the patient died from progressive disease. LG is a rare disease and a difficult diagnosis in the routine clinical practice. This report emphasises that, LG should be considered especially when there are migratory nodules of varying sizes in lungs.
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PMID:Migratory nodules in the lung: lymphomatoid granulomatosis. 1269 Nov 64

Lymphadenopathy is a common clinical finding and is frequently benign. Warning signs suggestive of a malignant etiology include lymph nodes >2 cm in size, supraclavicular location, and generalized lymphadenopathy associated with hepatosplenomegaly or systemic symptoms. A metastatic solid tumor is always in the differential diagnosis of localized lymphadenopathy, particularly in older individuals. In the case of more generalized lymphadenopathy, in addition to the more common lymphomas, benign etiologies as well as benign and atypical lymphoproliferative disorders need to be considered. Benign etiologies of lymphadenopathy can include infections, autoimmune disorders, drug hypersensitivity reactions, sarcoidosis, and amyloidosis. Rare but benign lymphoproliferative disorders include Kikuchi's disease, Rosai-Dorfman disease, and progressive transformation of germinal centers. Atypical lymphoproliferative disorders that bear close surveillance for evolution to a more aggressive malignancy include Castleman's disease, lymphomatoid granulomatosis, and lymphomatoid papulosis. Previously considered in this category but now classified as a true lymphoma is angioimmunoblastic lymphadenopathy with dysproteinemia. Physicians need to be aware of all of these disorders when evaluating suspicious lymphadenopathy, while also considering the more common lymphomas and leukemias.
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PMID:Clinical mimics of lymphoma. 1695 99

Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease. We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan revealed bilateral pulmonary nodules predominantly in lower lobes and peripheral lung fields. Laboratory exams showed pancytopenia. Skin biopsy was done, and histopathological examination and immunohistochemistry evaluation confirmed lymphomatoid granulomatosis. He was treated with steroid and cyclophosphamide but succumbed by neurologic involvement.
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PMID:Lymphomatoid granulomatosis with splenomegaly and pancytopenia. 2067 11

Griscelli syndrome type 2 (GS2) is a rare autosomal-recessive disorder associated with a RAB27A gene mutation, and clinically manifesting as hypopigmentation, disseminated chronic encephalitis, and severe immunological disorders characterized by an accelerated hematological phase, also referred to as hemophagocytic syndrome (HS), or hemophagocytic lymphohistiocytosis (HLH). The authors report the diagnosis of GS2 in an 11-year-old girl with hypopigmentation, immunodeficiency, hepatosplenomegaly, severe neurological impairments, and fatal multiorgan failure. In this patient a diagnosis of pulmonary lymphomatoid granulomatosis (LG), an Epstein-Barr virus (EBV)-related lymphoproliferative disorder, was established from radiological and histological findings. Although EBV-related malignancies are common in immunocompromised patients, this is the first report of a diagnosis of pulmonary LG in a patient with GS2.
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PMID:Pulmonary lymphomatoid granulomatosis in Griscelli syndrome type 2. 2211 99

Lymphomatoid granulomatosis (LYG) is an extremely rare angio-centric and angio-destructive B-cell lymphoproliferative disease. Driven by Epstein-Barr virus (EBV), LYG predominantly involves the bilateral lungs. Commonly presenting as multiple nodules in the lung, pulmonary LYG can masquerade as various infectious diseases, vasculitis, lung cancer, or other metastatic neoplasm. It is difficult to be diagnosed and is always neglected by clinicians. No standardized therapeutic regimens for LYG has been established yet now. Hemophagocytic lymphohistiocytosis (HLH), a life-threatening condition caused by abnormal activation of macrophages and T-cells, is characterized by fever, hepatosplenomegaly, pancytopenia, hypercytokinemia, and the presence of hemophagocytosis within the bone marrow, liver, spleen, or other lymphatic tissue. We herein report a 55-year-old man with recurrent fever, severe jaundice, and multiple high-density opacities and nodules in both lungs, who was finally diagnosed with pulmonary LYG (Grade 3) manifested with secondary HLH. Administration of HLH-1994 protocol led to the rapid control of the symptoms caused by HLH. Rituximab-based combination therapy was useful yet LYG (Grade 3) progressed rapidly. This case demonstrates that tissue biopsy is essential for early pathological diagnosis and effective treatment of LYG.
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PMID:Pulmonary Lymphomatoid Granulomatosis With Hemophagocytic Lymphohistiocytosis as the Initial Manifestation. 3206 34


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