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

Immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.
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PMID:Immunoblastic lymphadenopathy with purpura and cryoglobulinemia. 30 Oct 9

Immunoblastic lymphadenopathy was first described in the german literature in 1975 by Lennert as "Lymphogranulomatosis X." The disease is characterized by generalized lymphadenopathy, hepatosplenomegaly, dysproteinemia, fever and hyperergic reactions such as pruritus, skin rush and eosinophilia. The first ORL occurrence of the disease in Waldeyer's ring and cervical lymph nodes is reported. The histologic picture is characterized by a vascular proliferation with immunoblasts, plasma cells and interstitial amorphous acidophilic material but without Sternberg cells. The origin of the disease from primary malignant neoplasia or hyperergic reactions has not yet been defined. Although prognosis is uncertain and therapy limited, the best management involves small doses of corticoids, supplemented by antibiotics.
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PMID:[Head and neck manifestations of lymphogranulomatosis X (author's transl)]. 70 Nov 5

Immunoblastic lymphadenopathy, although it resembles Hodgkin's disease, is a distinct, hyperimmune disorder apparently of the B-cell system. In 32 cases, it was characterized by a morphologic triad: proliferation of arborizing small vessels; prominent immunoblastic proliferations; and amorphous acidophilic interstitial material. Clinically, it is manifested by fever, sweats, weight loss, occasionally a rash, generalized lymphadenopathy and often hepatosplenomegaly. There is a consistent polyclonal hyperglobulinemia and often hemolytic anemia. The course of the disease is usually progressive, with a median survival of 15 months in 18 fatal cases. The cellular proliferation appears benign morphologically in the pretherapy biopsies and in 10 of 12 available autopsy cases. In three cases the process evolved into a lymphoma of immunoblasts, immunoblastic sarcoma. The basic process appears to be a non-neoplastic hyperimmune proliferation of the B-cell system involving an exaggeration of lymphocyte transformation to immunoblasts and plasma cells that may be triggered by a hypersensitivity reaction to therapeutic agents.
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PMID:Immunoblastic lymphadenopathy. A hyperimmune entity resembling Hodgkin's disease. 107 47

Immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma is a distinct peripheral T-cell lymphoma, which closely resembles angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) and/or IBL, but is characterized by focal or sheet-like proliferation of immunoblasts and pale cells of T-cell nature. In this report, 36 patients with IBL-like T-cell lymphoma were analyzed. The disease is clinically characterized by generalized lymph node swelling, hepatosplenomegaly, fever, skin rash, polyclonal hypergammaglobulinemia, marked male predominance, predilection for the elderly, and poor prognosis. There was no association with human T-cell leukemia virus type I or human immunodeficiency virus. IBL-like T-cell lymphoma may be divided into two categories (CD4+ type and CD8+ type) by surface marker analysis. It can also be divided into three categories on the basis of the histologic findings of distribution of morphologically recognizable tumor cells: nine cases of "inconspicuous type," six cases of "patchy type," and 21 cases of "diffuse type." Two cases of "inconspicuous type" converted later to "diffuse type." DNA hybridization analyses in the ten recent cases revealed that three of four "inconspicuous types" and five of six "diffuse types" showed clonal rearrangement of T-cell receptor-beta chain gene without rearrangement of immunoglobulin heavy chain gene, providing strong evidence for clonal proliferation of T cells.
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PMID:Clinicopathologic, immunophenotypic, and immunogenotypic analyses of immunoblastic lymphadenopathy-like T-cell lymphoma. 304 80

The present study evaluates the significance of clinical and histologic features in 16 cases of angioimmunoblastic lymphadenopathy (AIL). The disease observed in the elderly is characterized by an acute onset of constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, and immunologic abnormalities. The clinical course is rather short, and autopsied cases show severe infections complications. In the present study, two histologic types were identified: 1) Angio-cellular (A-C) type: the lymph nodes contained lymphocytes, immunoblasts, histiocytes, eosinophils and/or plasma cells. 2) Immunoblastic and plasma cell (I & P) type or lymphocyte depleted (LD) type; the lymph node contained mainly immunoblasts and plasmocytoid cells. Serum protein was examined in 12 cases. Dysproteinemia was noted in 5 of 6 cases of A-C type and in 5 of 6 cases of I & P type. There is a definite relationship between histologic types and survival periods. The A-C type is an expression of slight depletion of lymphocytes and the I & P or LD type represents marked depletion of lymphocytes. AIL has malignant potentiality, though it resembles graft-vs-host reaction in histology, the wide-spread involvement of tissues and clinical features. An immunologic investigation on 3 cases suggests that the disease is based on B-cell disorder.
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PMID:Angioimmunoblastic lymphadenopathy: light and electron microscopic studies. 697 56

Immunoblastic lymphadenopathy presents with fever, malaise, cough, dyspnea, lymphadenopathy, hepatosplenomegaly, hypergammaglobulinemia, interstitial pulmonary infiltrates, mediastinal adenopathy, and effusions. The diagnosis is made on the basis of lymph node biopsy. Based on the course in our patients and the cases presented in previous reports, a suggested program of treatment for immunoblastic lymphadenopathy includes administration of moderate doses of steroids initially, with a subsequent increase to a higher dosage if desired improvement does not occur. Chemotherapy with three drugs, cyclophosphamide, vincristine, and prednisone, is indicated if remission using steroids fails. The diagnostic dilemma of whether pulmonary infiltrates are due to the disease itself or to pulmonary infection or to cytotoxic changes from chemotherapy always exists and often requires specimens from either transbronchoscopic or open-lung biopsy for definitive diagnosis and treatment.
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PMID:The lung in immunoblastic lymphadenopathy. 727 80