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)

A 14-year-old boy presented with fever, anemia, hepatosplenomegaly, generalized lymphadenopathy and nephrotic syndrome. Lymph node biopsy showed angiofollicular lymph node hyperplasia (generalized Castleman's disease) of the plasma cell type. Kidney biopsy showed membranoproliferative glomerulonephritis type 1. Complete remission was achieved with corticosteroid treatment and repeat kidney biopsy 22 months later showed complete resolution of the renal pathology. The association between membranoproliferative glomerulonephritis and multicentric angiofollicular lymph node hyperplasia, plasma cell type, has not previously been reported.
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PMID:Membranoproliferative glomerulonephritis associated with multicentric angiofollicular lymph node hyperplasia. Case report and review of the literature. 129 48

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

A patient presenting with predominantly gastrointestinal symptoms and a history of myocardial infarction was found to have ascites, hepatosplenomegaly, para-aortic lymphadenopathy, thrombocytosis, and a paraproteinaemia. A jejunal biopsy specimen showed lymphangiectasia and histology of the spleen and lymph nodes showed angiofollicular hyperplasia or Castleman's disease of the hyaline vascular type. This association has not previously been described and, moreover, systemic symptoms are unusual in this variant of Castleman's disease.
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PMID:Intestinal lymphangiectasia associated with angiofollicular lymph node hyperplasia (Castleman's disease). 174 Feb 68

A clinicopathologic analysis of 16 cases of multicentric angiofollicular lymph node hyperplasia (MAFH) was performed. Histologically, the disease was characterized by recognizable lymph node architecture that was at least partially intact, by paracortical hyperplasia with prominent vascular proliferation, and by numerous evenly distributed, apparently benign germinal centers of various types, usually including some typical hyaline-vascular centers. At the onset of the disease, 12 patients had the plasma cell (PC) type of MAFH, three patients had the hyaline-vascular (HV) type, and one patient presented with PC and HV types at separate sites. Transitions between the PC and HV types were observed in two cases. Immunologic studies demonstrated polyclonal populations of plasma cells in the lymph nodes of all patients and the absence of suppressor T lymphocytes in the one patient tested. Clinically, the patients had constitutional symptoms, multicentric lymphadenopathy, hepatosplenomegaly in many cases, and abnormal laboratory findings, including anemia, polyclonal hypergammaglobulinemia, and bone marrow plasmacytosis. The 16 patients were placed in four different clinical groups based on presentation and course: stable disease, chronic relapsing disease, aggressive disease, and development of malignant lymphoma. Ten of the 16 patients died (median survival, 26 months; range, eight to 170 months). Multicentric angiofollicular lymph node hyperplasia appears to be a variant of classic angiofollicular lymph node hyperplasia (Castleman's disease) and is associated with significant morbidity and mortality.
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PMID:Multicentric angiofollicular lymph node hyperplasia: a clinicopathologic study of 16 cases. 257 15

A 38-year-old black man with giant lymph node hyperplasia (GLH), osteoblastic lesions, and the POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) was treated at the University of Chicago Hospitals. The patient had hepatosplenomegaly and generalized peripheral lymphadenopathy. Endocrinologic abnormalities included decreased testosterone with elevated luteinizing hormone and follicle-stimulating hormone, as well as hyperprolactinemia and possible hypothyroidism. Biopsy of a right femoral lymph node revealed GLH, and an osteoblastic pelvic lesion showed a marked lymphoplasmacytic infiltrate. By immunohistochemical techniques, plasma cells in the lymph node and osteoblastic lesion were polyclonal. A polyclonal hypergammaglobulinemia was present. The lymph node T-lymphocyte population showed a decreased helper-to-suppressor cell ratio. Other findings included thickening of the skin, finger clubbing, and anasarca. A severe sensory-motor polyneuropathy was the major factor contributing to the patient's death. The association of GLH, osteoblastic bone lesions, and the POEMS syndrome has been noted previously in Japan; however, the authors are unaware of reports on Western patients who had this combination of clinical and laboratory findings.
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PMID:Giant lymph node hyperplasia with osteoblastic bone lesions and the POEMS (Takatsuki's) syndrome. 389 Oct 64

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

Castleman's disease (also called giant lymph node hyperplasia or angiofollicular lymph node hyperplasia) is a clinicopathological entity of unknown etiology. Two histologic patterns of lymph nodes are classically recognized: the hyaline-vascular and plasma-cell variants. Recently, multicentric Castleman's disease has emerged as a separate clinical entity manifested primarily by generalized lymphadenopathy and systemic manifestations, such as thrombocytopenia, hemolytic anemia, hepatosplenomegaly, altered liver function tests, central nervous system alterations, and autoimmune manifestations. A number of renal alterations have been described in association with the two pathological variants of Castleman's disease, but thrombotic microangiopathy has been previously reported only once in a patient with Castleman's disease. No renal biopsy was performed in that patient, although there was evidence of renal dysfunction. We report two cases of biopsy-proven renal thrombotic microangiopathy associated with multicentric Castleman's disease. In addition to having lymph node pathology characteristic of Castleman's disease, both patients presented with generalized lymphadenopathy and systemic manifestations, including acute renal failure, hypergammaglobulinemia, anemia, thrombocytopenia, and hypoalbuminemia. Autoantibodies were present in both patients, including antiphospholipid antibodies in one patient. The renal biopsies, examined by light, immunofluorescence, and electron microscopy, were diagnostic for renal thrombotic microangiopathy. The simultaneous development of two rather uncommon syndromes, multicentric Castleman's disease and renal thrombotic microangiopathy, suggests a possible link between Castleman's disease and renal thrombotic microangiopathy. Furthermore, we propose that the production of autoantibodies, in particular antiphospholipid antibodies, may lead to the development of thrombotic microangiopathy in some patients with multicentric Castleman's disease.
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PMID:Renal thrombotic microangiopathy associated with multicentric Castleman's disease. Report of two cases. 766 Dec 75

Multicentric Castleman's disease (MCD), or multicentric angiofollicular lymph node hyperplasia, is an uncommon lymphoproliferative disorder which typically present with constitutional symptoms, multicentric lymphadenopathy, hepatosplenomegaly, effusions, and ascites. We describe a patient with several novel manifestations of MCD: sicca syndrome, lacrimal and salivary gland enlargement, cardiomyopathy, and palmar and plantar rash. Treatment of MCD with chlorambucil and prednisone was effective in the short term followup of this patient. MCD merits consideration in patients with lymphadenopathy and multisystem disease, including sicca syndrome, heart failure or rash.
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PMID:Presentation of multicentric Castleman's disease with sicca syndrome, cardiomyopathy, palmar and plantar rash. 816 21

Multicentric angiofollicular lymphnode hyperplasia (multicentric Castleman's disease) may be associated with acute phase reaction and several autoimmune features. Since lymphadenopathy is a common feature in connective tissue disease, a clear distinction between the different disease entities may be difficult. We describe a 26-year-old male patient with predominant cervical lymphadenopathy, hepatosplenomegaly and polyserositis, diagnosed as collagen disease. He showed several autoimmune features including autoimmune haemolytic anaemia, cryoglobulinaemia, positive antinuclear and anti smooth muscle antibodies, serum immune complexes and a sensorimotor polyneuropathy. Under immunosuppressive therapy with prednisolone and azathioprine, only partial remission was achieved. Repeated lymph node biopsy together with the clinical features led to the diagnosis of multicentric Castleman's disease in this patient nine years later. Interleukin-6 (IL-6) seems to play an important role in the pathogenesis of clinical and serum biochemical features in patients with Castleman's disease.
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PMID:Autoimmune findings resembling connective tissue disease in a patient with Castleman's disease. 913 32

Kaposi's sarcoma-associated herpesvirus (KSHV; also known as human herpesvirus 8 [HHV-8]) is a herpesvirus linked to the development of Kaposi's sarcoma (KS), primary effusion lymphoma, and a proportion of Castleman's disease. KSHV encodes viral interleukin-6 (vIL-6), which is structurally homologous to human and murine IL-6. The biological activities of vIL-6 are largely unknown. To gain insight into the biology of vIL-6, we expressed vIL-6 in murine fibroblasts NIH3T3 cells and inoculated stable vIL-6-producing clones into athymic mice. vIL-6 was detected selectively in the blood of mice injected with vIL-6-expressing clones. Compared with controls, vIL-6-positive mice displayed increased hematopoiesis in the myeloid, erythroid, and megakaryocytic lineages; plasmacytosis in spleen and lymph nodes; hepatosplenomegaly; and polyclonal hypergammaglobulinemia. vIL-6-expressing NIH3T3 cells gave rise to tumors more rapidly than did control cells, and vIL-6-positive tumors were more vascularized than controls. Vascular endothelial growth factor (VEGF) was detected at higher levels in the culture supernatant of vIL-6-expressing cells compared with controls, and immunohistochemical staining detected VEGF in spleen, lymph nodes, and tumor tissues from mice bearing vIL-6-producing tumors but not control tumors. Thus, vIL-6 is a multifunctional cytokine that promotes hematopoiesis, plasmacytosis, and angiogenesis. Through these functions, vIL-6 may play an important role in the pathogenesis of certain KSHV-associated disorders.
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PMID:Angiogenesis and hematopoiesis induced by Kaposi's sarcoma-associated herpesvirus-encoded interleukin-6. 1036 Oct 99


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