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 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

BALB/c mice neonatally injected with (BALB/c X C57BL/6)F1 hybrid spleen cells develop host-versus-graft disease (HVGD) with immunopathological features characteristic of either systemic lupus erythematosus (SLE), or immunoblastic lymphadenopathy (IBL) in man. HVGD mice manifest polyclonal hypergammaglobulinemia with various autoantibodies, generalized lymphadenopathy, hepatosplenomegaly, immune complex glomerulonephritis and evolve in the end to malignant lymphoma. The necessary prerequisite for HVGD induction between donor and host can be summarized as follows: Histoincompatibilities in the H-2 region between donor and host are needed; predominant F1 donor cells needed for HVGD induction are, if not sole, steroid resistant, nylon wool nonadherent and Thy-1 positive T cells; the role of donor T cells is not only to present H-2 complex to the host, but also to interact with and proliferate in the host; strain differences are found for the susceptibility of HVGD induction in the host. It has been found that HVGD evolves to a malignant lymphoma of host T cell origin. The HVGD mouse model may, therefore, contribute to the understanding of the cell to cell interactions at work in the pathogenesis of IBL, as well as SLE, in man.
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PMID:Autoimmune disease and malignant lymphoma associated with host-versus-graft disease in mice. 408 49

Angioimmunoblastic lymphadenopathy is a disease characterized systemic symptoms, lymphadenopathy, hepatosplenomegaly and polyclonal hypergammaglobulinemia. Hematologic abnormalities are common, especially anemia, which is often Coombs positive, and lymphocytopenia. This report cites a case of angioimmunoblastic lymphadenopathy associated with selective hypoplasia of the myeloid line and normal maturation of all other marrow-derived cell lines. Possible relationships of this finding to the immunologic abnormalities associated with angioimmunoblastic lymphadenopathy are discussed.
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PMID:Angioimmunoblastic lymphadenopathy with associated selective myeloid hypoplasia. 670 2

This is the first known report of a case of angioimmunoblastic lymphadenopathy, with dysproteinemia (AILD) in childhood which evolved toward a spontaneous remission. The disease had an acute onset with generalized lymphadenopathy, hepatosplenomegaly, high-grade fever and polyclonal hypergammaglobulinemia. The lymph nodes met all of the histologic criteria required for diagnosis as established by Frizzera et al. It is emphasized that AILD should be taken into consideration in the differential diagnosis of lymphadenopathy in childhood.
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PMID:Angioimmunoblastic lymphadenopathy with dysproteinemia: report of the first case in childhood evolving toward spontaneous remission. 728 61

The clinical features of 13 patients with angioimmunoblastic lymphadenopathy were analyzed to determine prognostic factors and response to therapy. Eleven patients presented with sudden onset of fever, weight loss, generalized lymphadenopathy, and hepatosplenomegaly. Laboratory features included autoimmune hemolytic anemia and polyclonal hypergammaglobulinemia. Pulmonary involvement was seen in six cases and skin rash in four. Two patients had localized lymphadenopathy without systemic symptoms. Both are alive at 5.5 and 2.5 years, respectively, after diagnosis, although the latter patient has required intermittent prednisone for recurrent lymphadenopathy. An additional patient is alive on treatment for months following diagnosis. The remaining ten have died, nine of sepsis and one of cerebral hemorrhage. The immunosuppression and myelosuppression of combination chemotherapy may have hastened their deaths. An individualized, conservative treatment approach is recommended.
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PMID:Angioimmunoblastic lymphadenopathy: clinical spectrum of disease. 729 97

An eight-months-old infant presenting with anemia, hepatosplenomegaly, hypoalbuminemia and polyclonal hypergammaglobulinemia was finally found to be affected by visceral leishmaniasis, after having suspected oncohaematologic problems. Diagnosis was confirmed by bone marrow aspiration. Complete recovery was achieved with antimonial N-methylglucamine (Glucantim) administered IM.
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PMID:[Anemia and hepatosplenomegaly: search for the protozoon?]. 788 58

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rare lymphoproliferative disorder characterized by diffuse lymphadenopathy, fever, hepatosplenomegaly, hemolytic anemia, and polyclonal hypergammaglobulinemia. Morphologically, the involved lymph nodes demonstrate complete effacement of the normal architecture, prominent neovascularization and infiltration by immunoblasts and plasma cells. Other terms that have been used to describe this entity include diffuse plasmacytic sarcomatosis, immunoblastic lymphadenopathy, lymphogranulomatosis X, and immunologic aberrations in idiopathic reticulosis. Initially, AILD was thought to be a disease of B-cell origin that represented reactive immune response to unknown stimulus and high potential for malignant transformation. It is now evident that AILD in 80% of cases follows an aggressive course with short median survival, especially, if complete response with chemotherapy is not achieved. Immunologic and molecular studies have demonstrated that the majority of AILD cases are T-cell clonal disorders. Despite the numerous reports on the role of Epstein-Barr virus in this disorder, it is unknown whether the presence of this virus is associated with the immune defect that accompanies AILD, or whether it is a pathogenetic factor. In contrast to non-Hodgkin's lymphomas, a stage is not usually assigned to the patient since the disease is systemic in nature, subsequently, parameters such as extent of disease and tumor bulk used to identify high-risk patients with non-Hodgkin's lymphomas, do not appear to correlate with disease activity or prognosis in AILD. Treatment of AILD has been unsatisfactory, with approximately 25% of patients achieving complete and sustained remission when combined chemotherapy agents are used. This article is devoted to a discussion of the different manifestations, suggested pathogenesis, and treatment of AILD.
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PMID:Angioimmunoblastic lymphadenopathy with dysproteinemia: emphasis on pathogenesis and treatment. 985 98

Angioimmunoblastic T-cell lymphoma (or angioimmunoblastic lymphadenopathy with dysgammaglobulinemia [AILD]) was originally considered to be an abnormal immune reaction in which reactive follicles with germinal centers (GCs) are usually absent. When hyperplastic GCs are present along with an angioimmunoblastic reaction, the lesion has been interpreted as a benign hyperimmune reaction. We report seven patients with angioimmunoblastic T-cell lymphoma (AITL) who initially had hyperplastic GCs, shown to be malignant lymphoma by further studies and clinical follow-up. Clonal T-cell populations were observed in all specimens evaluated, and sequential biopsies showed histologic progression to typical AITL in two patients. Clinical presentation was characterized by generalized lymphadenopathy of acute onset, constitutional symptoms, hepatosplenomegaly, skin rash, and polyclonal hypergammaglobulinemia in five patients; regional adenopathy preceded generalized adenopathy in two patients. Five patients had rapid progression of disease, and three patients whose treatment was delayed due to inadequate evidence to diagnose lymphoma died of infection. The initial biopsy findings of each patient were similar and showed angioimmunoblastic proliferation, hyperplastic GCs with ill-defined borders, and interfollicular tingible-body macrophages. These GCs differed from occasional residual follicles of typical AITL in that the GCs were enlarged and hyperplasia of follicular dendritic cells was not seen. Diagnostic clear cells were not observed. Apoptotic bodies were markedly increased and bcl-2+ lymphocytes were sparse compared with typical AITL. Results of in situ hybridization for Epstein-Barr virus were positive in each case. We conclude that hyperplastic germinal centers with ill-defined borders and frequent interfollicular tingible-body macrophages occur in a histologic variant of AITL that is necessary to recognize for early diagnosis and treatment.
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PMID:Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplastic germinal centers. 963 Jan 71

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

Angioimmunoblastic lymphadenopathy (AILD) is a rare disorder characterised by generalised lymphadenopathy, fever, hepatosplenomegaly, immune hemolytic anemia and polyclonal hypergammaglobulinemia. We report the occurrence of histology-proven AILD in a patient who had received ciprofloxacin. We suggest that this drug may be added to the list of agents susceptible to elicit AILD.
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PMID:Angioimmunoblastic lymphadenopathy following ciprofloxacin administration. 1215 41


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