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)

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

Eight elderly women with primary non-Hodgkin's lymphoma were treated with a combination of surgery, chemotherapy and radiation with excellent outcome. Clinically, the patients presented with thyromegaly and did not have lymphadenopathy or hepatosplenomegaly. On histological examination, the lymphomas resembled diffuse follicle-centre cell tumours. All patients showed extension beyond the thyroid capsule and lympho-epithelial lesions; 3 displayed kappa-light-chain restriction and 2 lambda-monoclonality. Thyroid lymphomas fall into the spectrum of mucosa-associated lymphomas, which have a better prognosis than their equivalent nodal-based counterparts.
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PMID:Primary thyroid lymphomas. A clinicopathological study of 8 cases. 223 90

Subchronic and chronic toxicities of hexachlorobenzene (HCB) were studied in both sexes of Swiss mice, Syrian golden hamsters and Sprague-Dawley rats, at dietary dosages of 0, 100 and 200 ppm (mice), and 0, 200 and 400 ppm (hamsters and rats) for 90 days. At day 91, 25/50 animals in each of 18 groups were killed for histology studies. The rest were killed at 6-week intervals until the study was ended. Marked hepatosplenomegaly, enlarged thymuses and lymph nodes, or swollen and granular-looking renal cortices with depressions or nodulary areas were commonly observed. Dose- and sex-dependent progressive changes included toxic-degenerative hepatitis, chronic cirrhosis, hepatomas, bile-duct adenomas and a few hepatocarcinomas in older animals. A generalized lymphohaematopoietic response led to thymic, splenic and nodal lymphosarcomas, especially in female mice. Toxic-tubular nephritis with cortical infarcts developed into regenerative foci and renal adenomas in low incidences. Liver lesions were more prominent in females, while renal changes were most common in male rats. HCB was retested in both sexes of rats at oral doses of 0, 75 and 150 ppm for up to 2 years. At the start, each group contained 94 rats, and four randomly selected rats were killed at weeks 0, 1, 2, 3, 4, 8, 16, 32, 48 and 64 for microscopy. Progressive liver lesions started as hyperaemia and degenerations (4 weeks), and developed into toxic hepatitis, cirrhosis and formation of pre- and neoplastic foci (36 weeks), with hepatomas, bile-duct adenomas and hepatocellular carcinomas (64 weeks) in very high incidences in females and renal adenomas in male rats.
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PMID:Oncogenicity of hexachlorobenzene. 359 33

Peripheral lymph node enlargement was found in 14 of a series of 132 feline lymph node biopsy specimens. Six of nine cats tested had antibodies for feline leukemia virus (FeLV). Half of the cats were clinically normal while the remainder had fever, lethargy, anorexia, and hepatosplenomegaly. There was severe distortion of lymph nodal architecture with variable loss of discernible follicles and sinuses. Histiocytes, lymphocytes, immunoblasts, and plasma cells were present in expanded paracortical regions which encroached on, and occasionally effaced, lymphoid follicles. Postcapillary venules were numerous and prominent throughout the paracortex. The lymphadenopathy was most commonly transient (86% of cases) with subsequent development of lymphoma in one cat. Lymph nodes from seven kittens with experimental FeLV infection were compared with spontaneously enlarged lymph nodes; four of seven had B and T lymphocyte hyperplasia with normal nodal architecture. Three had partial loss of nodal architecture as a result of expanded paracortical regions populated largely by histiocytes and lymphocytes. Proliferation of postcapillary venules was not prominent in nodes from FeLV-infected cats. The cause of spontaneous lymph node hyperplasia of young cats was not determined. However, the similarity of lesions to those of kittens with experimental FeLV infection and the association with FeLV by serologic tests in six of nine cats suggest that this retrovirus may be involved in the pathogenesis of the lesion.
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PMID:Distinctive peripheral lymph node hyperplasia of young cats. 375 Jul 32

This study is based on an analysis of the morphologic, clinical, and laboratory findings in 26 patients whose pretherapy lymph node biopsies showed some, but not all, of the diagnostic features of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD). Partial or complete effacement of nodal architecture by a diffuse lymphoplasmacytic and immunoblastic proliferation was a constant histologic finding. In contrast to the findings in AILD, lymphocytic depletion and pronounced arborizing vascular proliferation were often lacking. Clinically, many of the patients had fever, sweats, weight loss, skin rashes, generalized lymphadenopathy, hepatosplenomegaly, and, in some cases, pulmonary infiltrates. Of the 26 patients, 23 had clinical and/or laboratory evidence of autoimmune disease or immune complex disease. In 12 patients (Group I--idiopathic), various autoantibodies or immune complexes were demonstrable, but these patients did not manifest a well-defined immunologic disease or syndrome. In 11 patients (Group II--secondary), the lymphadenopathy occurred secondary to a well-defined, clinically recognized immunologic disease. Three patients (Group III) had neither a well-defined autoimmune disease nor demonstrable autoantibodies, but two of them had a history of exposure to antibiotics. We suggest that patients whose lymph nodes have the morphologic features described here frequently have an autoimmune disorder, and that the pathogenesis of this clinicopathologic picture is probably related to a deficiency in suppressor T-cell function which results in an unopposed proliferation of B cells with autoantibody formation and polyclonal gammopathy. Our observations should stimulate clinicians to consider the possibility of an autoimmune pathogenesis for a lymphadenopathy in which a florid lymphoplasmacytic and immunoblastic proliferation similar to that observed in AILD is demonstrated, even though the sections may not meet all the histologic criteria reported for the diagnosis of AILD. Clinical and laboratory investigations necessary to confirm the presence of autoimmunity are indicated in these cases. Moreover, since there is evidence of genetic factors predisposing to autoimmune disease (17, 43), it would be important to investigate close relatives of patients whose lymph nodes showed the histologic changes described in this paper in prospective studies which include suppressor T-cell function, autoantibodies, HLA type of blood lymphocytes and chromosomal analysis. The median survival of the 23 patients with stigmata of autoimmune disease or immune complex disease was 36 months.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Atypical lymphoplasmacytic and immunoblastic proliferation in lymph nodes of patients with autoimmune disease (autoimmune-disease-associated lymphadenopathy). 638 57

This report describes the nodal and extranodal lesions observed in 15 patients with a generalized disorder that had been histologically diagnosed as Castleman's disease. The disorder was characterized by severe constitutional symptoms, constant involvement of multiple peripheral lymph nodes, and frequent hepatosplenomegaly, in association with clinical and laboratory features reminiscent of a "collagen disease." The clinical course was chronic, with remissions and exacerbations in seven patients, and aggressive and fatal in eight. The material examined included multiple lymph node biopsies, four surgical specimens of spleen, one open lung biopsy, and material from four autopsies. The diagnostic morphological findings were observed in the nodes and were represented by the following histologic triad: diffuse marked plasmacytosis, from the medulla to the subcapsular areas; prominence of the germinal centers; and good preservation of the architecture. One variant of this basic pattern featured abundant immunoblasts and blood vessels. The process appears to be a systemic reactive proliferation of B-lymphocytes, perhaps resulting from faulty immune regulation. Morphologic similarities indicate a relationship between this multicentric disorder and Castleman's disease of plasmacellular type. However, there are distinct differences between them in clinical presentation and evolution, and, consequently, in therapeutic approach.
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PMID:A systemic lymphoproliferative disorder with morphologic features of Castleman's disease. Pathological findings in 15 patients. 683 32

A 4-year-old girl presenting with vomiting, abdominal pain, and renal failure was found to have gross hepatosplenomegaly, a renal mass, and bilateral pleural effusions. A diagnosis of acute lymphoblastic leukaemia (ALL) was suggested by a peripheral white cell count (WCC) of 119,000 x 10(6)mm3, 57% blasts, 22% lymphocytes, and confirmed by bone marrow examination. Lymphocyte surface marker studies at diagnosis enabled classification as a T-ALL, with a significant proportion of the T cells also bearing receptors for the third component of complement (C3). Seventy-two percent of the peripheral blood mononuclear cells reacted with anti-Ia monoclonal antibody (FMC44), and a smaller proportion (25%) carried receptors for the Fc portion of IgG. The T-classification of this ALL was verified at central nervous system (CNS) relapse and at a subsequent nodal relapse. Double-marker studies on cells from the infiltrated lymph node prepared in suspension confirmed the presence of Ia-positive T cells. The Ia marker is usually a useful discriminant between T and non-T cells in normal and ALL cell populations. The case described here highlights the need for a panel of markers to be used in classification of childhood ALL and supports the suggestion that there is a distinct subtype of Ia-positive T-ALL.
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PMID:Childhood T-cell acute lymphoblastic leukaemia expressing "Ia-like" antigen:" a case report. 698 Oct 53

We present two pseudolymphoma occurring 8 days and 1 month after carbamazepine introduction. Both patients present fever, rash, generalized lymphadenopathy and hepatosplenomegaly in the second case. Hematologic abnormalities included anemia, eosinophilia, hepatic cytolysis. Histologic evaluation of a lymph node biopsy specimen demonstrated near-total effacement of the nodal architecture mimicking a lymphoma. Gene rearrangement studies proved the benign nature of the proliferation. Carbamazepine-induced lymphoproliferative disorders are relatively rare with only 38 observations published. The pathogenesis is uncertain. Immune dysregulation is probable. Morphologic and immunophenotypic data must be completed by gene rearrangement studies. Corticoid therapy is useless. The evolution is favorable after the cessation of carbamazepine.
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PMID:[Pseudolymphoma induced by carbamazepine. Apropos of 2 cases]. 774 Feb 34

Forty-six nodal T-cell lymphomas, classified according to the updated Kiel classification, were investigated for the presence of Epstein-Barr virus (EBV) DNA by polymerase chain reaction (PCR), EBER 1 and 2 (EBER 1/2) and latent membrane protein-1 (LMP-1) expression. A combination of RNA in situ hybridization and immunohistochemistry was used to establish the phenotype of the Epstein-Barr virus harbouring cells. In 21 of 45 cases Epstein-Barr virus DNA sequences could be detected with the polymerase chain reaction. In 15 cases (14 of 21 EBV PCR positive cases), EBER 1/2 positive cells could be demonstrated. As judged by morphology, EBER 1/2 expression was found in nonneoplastic and neoplastic lymphoid cells. Double staining revealed that more than 80% of the EBER 1/2 harbouring cells, lacked B-, T- or histiocytic markers, suggesting down regulation of T- and B-cell markers by Epstein-Barr virus. In eight of 15 cases some EBER 1/2 positive T-cells (CD3, CD45RO, CD43) morphologically resembling tumour cells were found. In nine of 14 cases tested EBER 1/2 positive non-neoplastic B-cells (CD20) were seen. Based on in situ hybridization results, four patterns of EBER 1/2 positive cells were found, i.e. single cells (< 1 per medium power field (mpf), n = 3), scattered (1-25/mpf, n = 4), clustered (26-100/mpf, n = 5) and diffuse (> 100/mpf, n = 3). In eight of 15 cases a clustered or diffuse pattern of EBER 1/2 positive cells was found and these lymphomas were therefore considered to be strongly associated with Epstein-Barr virus. In these lymphomas LMP-1 expression was found to be associated with an aggressive clinical course and hepatosplenomegaly.
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PMID:Detection of Epstein-Barr virus nucleic acid sequences and protein in nodal T-cell lymphomas: relation between latent membrane protein-1 positivity and clinical course. 831 34

Natural killer (NK)-like T cells are major histocompatibility complex-unrestricted cytotoxic T cells that are surface CD3-positive, express NK-cell antigens, and rearrange their T-cell receptor. Most neoplasms arising from this T-cell subpopulation have been a chronic lymphoproliferative disease referred to as T-large granular lymphocyte (LGL) leukemia. Only 10 NK-like T-cell lymphomas have been described in detail previously; this study presents the clinicopathologic features of six others and distinguishes these lymphomas from T-LGL leukemia. All patients presented with B-symptoms and often had marked hepatosplenomegaly without significant peripheral lymphadenopathy. Four of the six patients were immunosuppressed. All had CD3, CD8, CD56-positive tumors, presumably of hepatosplenic (n = 3), intestinal (n = 1), pulmonary (n = 1), or nodal (n = 1) origin. Three patients had lymphomatous bone marrow infiltrates, and four had peripheral blood involvement by neoplastic large lymphocytes, some of which had a blastic appearance or resembled virocytes. Azurophilic granules, ultrastructurally corresponding to cytoplasmic dense core and/or double density granules, were seen in all cases. T-cell clonality was shown in five tumors by Southern blot analysis, and three had abnormal karyotypes. Two untreated patients died 20 days after presentation, and three patients who received combination chemotherapy died within 5 months of presentation. One patient remains in complete remission 22 months after treatment. These findings suggest NK-like T-cell lymphomas are aggressive, are clinicopathologically distinct from T-LGL leukemia, and should be in the differential diagnosis of extranodal T-cell lymphoproliferations, including those in immunosuppressed patients. Furthermore, the LGL morphology, phenotype, and tissue distribution of some NK-like T-cell lymphomas suggest they arise from thymic-independent T cells of the hepatic sinusoids and intestinal mucosa.
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PMID:Natural killer-like T-cell lymphomas: aggressive lymphomas of T-large granular lymphocytes. 860 6


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