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 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

A patient with Lennert's lymphoma (malignant lymphoma with a high content of epithelioid histiocytes) is described. The case is unique in that hepatosplenomegaly was the presenting feature, the diagnosis was made on bone marrow biopsy and the disorder appears to have run a benign course.
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PMID:Lympho-epithelioid cellular lymphoma (Lennert's lymphoma): a case report. 87 Sep 95

A case is reported of a 72 year old man suffering from classical seropositive RA for 10 years. Two months before admission he experienced general illness, fever and itching. Lymph nodes enlargement and hepatosplenomegaly were found. Histologic features of lymph node biopsy were compatible with angioimmunoblastic lymphadenopathy and Lennert lymphoma. Rapidly progressive pulmonary deterioration followed with hilar invasion and honeycombing. Sternal punction, bone biopsy, bronchus biopsy and blind lung biopsy however, did not reveal lymphomatous invasion. On the contrary, lung biopsy, showed bronchiolitis obliterans, an often fatal, small airway disease whose connection with RA is discussed. Plasmapheresis induced a correction of leucopenia and thrombocytopenia. A fatal evolution evolved within six months. Autopsy revealed diffuse invasion by immunoblastic sarcoma. The clinicopathological entities angioimmunoblastic lymphadenopathy and Lennert lymphoma are referred to. The relationship with autoimmune disorders is stressed.
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PMID:Rheumatoid arthritis associated with bronchiolitis obliterans and immunoblastic sarcoma. 689 26