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

A transient dermatologic abnormality which gave a histologic picture compatible with malignant lymphoma is described. The clinical manifestations included diarrhea, hepatosplenomegaly, moderate lymph node enlargement without histopathological changes and evidence of involvement of the reticulo-endothelial system. Both the dermatologic and gastrointestinal disorders appeared approximately 1 year after discontinuation of treatment with diphenylhydantoin, which the patient had taken for 2 years. The possible association between the transient abnormality and the previous diphenylhydantoin therapy justifies the classification of this case as 'pseudolymphoma syndrome'.
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PMID:Psuedolymphoma syndrome. 14 19

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

Angio-immunoblastic lymphadenopathy (AIL) is a disease of unknown aetiology and pathogenesis. It has features of hyperimmunity, immune deficiency and can behave like a malignant lymphoma. An underlying abnormality of T-cell regulatory function has been proposed. We report ten patients with AIL followed prospectively and review 200 cases from the literature. As well as showing the typical features described in previous retrospective series namely: constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, skin eruptions, hypergammaglobulinaemia and characteristic lymph node histology; four patients had oedema with ascites or pleural effusions associated with hyponatraemia and hypoalbuminaemia. We have also observed low free thyroxine indices in three patients with elevated TSH levels in two, but without clinical features of hypothyroidism. Seven of the patients in this study were treated with prednisone and cyclophosphamide. Three achieved complete remission but only one patient has survived longer than two years. Failure to achieve complete remission has been associated with a 90 per cent mortality within one year of the onset of disease in the reports reviewed. Corticosteroids alone have produced only a 40 per cent rate of remission. The management of poor responders, non-responders and many relapse cases in unsatisfactory. More instensive chemotherapy is very hazardous, increasing the already high risk of severe infections, and is often unsuccessful. Histological features recently reported to represent co-existent immunoblastic lymphoma at diagnosis were recognized in three of our patients, two of which went on to develop definite lymphoma.
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PMID:Angio-immunoblastic lymphadenopathy: report of ten cases and review of the literature. 48 88

The clinical presentation and findings in 45 patients with lymphoma of the gastrointestinal tract occurring in an unselected population are described. Nineteen patients presented as emergencies with haemorrhage, perforation, pyloric stenosis or intestinal obstruction. Systemic symptoms, peripheral lymph node enlargement and hepatosplenomegaly were rare, and a preoperative diagnosis of lymphoma was seldom made. Endoscopy was superior to radiology in defining lesions in the stomach and duodenum. Lesions which were multicentric, or of unusual macroscopic appearance, were common in the small bowel, and these should raise the suspicion of lymphoma. Local and systemic recurrence was common, but was not always fatal. Patients with gastric lymphomas survived longer than those with intestinal disease.
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PMID:The presentation of gastrointestinal lymphoma: study of a population. 51 67

It is reported on a case of heavy chain disease in a 41-year-old man. It is a special form of the malignant lymphoma. The clinical picture of our patient distinguishes itself by an extraordinarily long course (more than 10 years enlargement of the spleen, since more than 5 years hypogammaglobulinaemia), abdominal malignant lymphomas with hepatosplenomegaly and perhaps connected with this intermittingly appearing acute epigastric complaints as well as the signs of a severe immunoinsufficiency. Homogenic Fc-(gamma)fragments are proved only in the serum in a concentration of 0.8 g/100 ml.
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PMID:[A case of Franklin disease--gamma heavy chain disease]. 81 23

Two patients are presented with lymphadenopathy, hepatosplenomegaly and infiltration of the bone marrow with plasmocytoid cells in whom an important heterogeneous hyperimmunoglobulinemia was detected (3.18 and 5.85 g/100/ml). Autopsy showed widespread poorly differentiated lymphocytic lymphoma with plasmocytoid differentiation in the first case and a well differentiated lymphocytic lymphoma with plasmocytoid differentiation involving lymph nodes, marrow, spleen, liver and kidney in the second case. High concentrations of IgM and IgG were detected in lymph node and spleen homogenates from this case. After splenectomy and cyclophosphamide the immunoglobulins decreased. It is suggested that both cases had a malignant lymphoma with plasmocytoid differentiation and polyclonal gammopathy.
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PMID:Malignant lymphoma with plasmacytoid differentiation and polyclonal gammopathy. 82 Jan 56

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

Twenty-six cases of histologically proven malignant histiocytosis are presented. Patient ages ranged from 20 months to 82 years, and a 10:3 male preponderance was seen. Clinical presentation is similar to that of malignant lymphoma and leukemia; histologic differentiation is required. The frequency of intrathoracic involvement at the time of presentation is similar to that of non-Hodgkin's lymphoma. Retroperitoneal lymph node invasion was readily seen on five of the eight lymphograms performed. Hepatosplenomegaly was a common finding, and three of the patients had evidence of bone involvement during the course of their illness. While there are no radiographic findings specific to malignant histiocytosis, the presence of hepatomegaly should suggest this possibility. The establishment of the correct diagnosis is important to guide choices in chemotherapy.
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PMID:Radiographic manifestations of malignant histiocytosis. 97 May 32

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

A 53-year-old male was diagnosed as having ataxic polyneuropathy associated with IgM-kappa monoclonal gammopathy in January 1988. Plasmapheresis and chemotherapy with chlorambucil and Melphalan-Prednisolone were effective for his neuropathy, but hemolytic anemia appeared in February 1989. The diagnosis of low-titer cold agglutinin disease (IgM-kappa) with anti-Pr2 specificity was made. Hemolytic anemia became refractory to high-dose corticosteroids, and fever, hepatosplenomegaly and severe pancytopenia appeared in January 1990. Bone marrow involvement of malignant lymphoma (mu, kappa) was found, and he died of pneumonia and gastrointestinal bleeding after the start of chemotherapy. Postmortem examination revealed a widespread infiltration of malignant lymphoma, diffuse, large cell (B-cell) type. Erythrophagocytic histiocytes also increased in bone marrow, liver, spleen and lymph nodes, as if there were hemophagocytic syndrome associated with lymphoma present. In addition to the high thermal amplitude of cold agglutinin in this case, the systemic activation of histiocytes induced by the development of malignant lymphoma may be responsible for progressive hemolysis and severe pancytopenia.
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PMID:[Anti-Pr2 cold agglutinin disease with polyneuropathy evolving to malignant lymphoma]. 133 64


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