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)

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

Two patients with immunoblastic lymphadenopathy had lymphadenopathy, hepatosplenomegaly, rash, dysproteinemia, constitutional symptoms, and typical morphologic findings or lymph node obliteration by immunoblasts and plasma cells, proliferation of aborizing vessels, and infiltration with amorphous, eosinophilic material. One patient had massive pulmonary infiltrates that responded to steroid therapy early in the course of the disease, but recurred and were found, at autopsy, to represent immunoblastic invasion of the lung. In both cases, a severe peripheral neuropathy developed during the course of the disease. One patient was addicted to dextroamphetamine for 20 years. Response to corticosteroids and cytotoxic therapy was initially rapid and complete, but refractoriness developed as seen in postmortem findings of extensive visceral involvement.
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PMID:Immunoblastic lymphadenopathy. A report of two cases. 30 47

Eight cases of angio-immunoblastic lymphadenopathy were recently diagnosed at the University Hospital of Geneva. The disease was characterized by generalized adenopathy, hepatosplenomegaly, fever, and sometimes skin rash. Six patients had polyclonal dysproteinemia and two displayed autoimmune disorders, e.g. Coombs' positive hemolytic anemia. Lymph node biopsy was a decisive pointer for the diagnosis. Four patients died, two of them from immunoblastic lymphoma. Four other patients are in remission, the longest follow-up being 91 months. Analysis of 213 cases from the literature shows the following features:--Males and females are equally affected, with a predominance after the age of 50.--The onset is characterized by constitutional symptoms (75%) and fever (65%), sometimes following exposure to drugs.--The signs are generalized adenopathy (91%), splenomegaly (66%), hepatomeagly (65%), and skin rash (46).--Polyclonal hypergammaglobulinemia (76%) is found, together with anemia (78%) which is often autoimmune in origin (58%).--The course is often unfavourable and the mortality is over 55%, largely due to opportunistic infections and/or development of immunoblastic lymphoma.
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PMID:[Angioimmunoblastic lymphadenopathy. 8 cases and review of the literature]. 35 86

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

We report a 47-year-old man diagnosed as angio-immunoblastic lymphadenopathy with dysproteinemia (AILD) with fibrosis of the bone marrow, lymph node, liver and spleen, and proliferation of epithelioid cells in lymph node. He was admitted to a hospital in May, 1980 because of general fatigue, cough, fever and systemic lymphadenopathy. The diagnosis of AILD was based on a biopsy of right cervical lymph node. His symptoms were improved but recurred with the addition of icterus and progressive pancytopenia with decrement of prednisolone. He was referred to our hospital in July, 1980 and his physical examination revealed generalized lymphadenopathy, icterus and hepatosplenomegaly. Hemogram showed pancytopenia, and needle biopsy of the bone marrow disclosed fibrosis. Sections from the lymph node showed AILD with proliferation of epithelioid cells. Administration of 60 mg/day of prednisolone improved the fever, lymphadenopathy and hepatosplenomegaly. However he died suddenly of acute respiratory failure on July 30. Autopsy showed fibrosis of bone marrow, lymph node, liver and spleen with infiltration of abnormal lymphocytes, and pulmonary aspergillosis.
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PMID:[Angio-immunoblastic lymphadenopathy with fibrosis of bone marrow, lymph node, liver and spleen, and proliferation of epithelioid cells in lymph nodes]. 221 92

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

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