Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Richter syndrome is an uncommon complication of chronic lymphocytic leukemia characterized by its transformation into diffuse histiocytic lymphoma. We present two documented cases of Richter syndrome and its radiographic manifestations, which have not previously been reported. These include hepatosplenomegaly, diffuse marked adenopathy, and involvement of the skeletal system. The diagnosis of Richter syndrome should be suggested when these radiographic findings occur with chronic lymphocytic leukemia.
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PMID:CT manifestations of Richter syndrome. 331 23

Three patients presented with liver granulomas and fever. An intensive investigation was initially negative. Corticosteroids were administered with dramatic clinical improvement. Ten months to 3 years later, upon tapering the corticosteroid dose, the initial manifestations reappeared. In two patients Hodgkin's lymphoma was discovered and in a third histiocytic lymphoma. Thus, the diagnosis of lymphoma should be considered in patients with idiopathic liver granulomas, fever, and hepatosplenomegaly without lymph node enlargement, who respond initially to steroid administration but relapse upon tapering the dose.
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PMID:Hodgkin's and non-Hodgkin's lymphomas masquerading as "idiopathic" liver granulomas. 646 11

Malignant histiocytosis (MH) and true histiocytic lymphoma (THL) are hematopoietic malignancies of the mononuclear phagocytic system distinguished from each other by clinical presentation and presumed cell of origin. THL present as a localized mass derived from the fixed tissue histiocyte which may or may not disseminate. MH originates from the circulating monocyte or tissue macrophage and is characterized by a syndrome of systemic symptoms, pancytopenia, adenopathy, hepatosplenomegaly, and wasting. The distinction between MH and THL is at times arbitrary and overlap exists between these syndromes. The clinicopathologic studies that defined these entities were performed prior to the development of immunophenotyping and other molecular techniques currently used to ensure proper classification of hematopoietic malignancies. Nine patients from the University of Minnesota originally diagnosed with MH were retrospectively analyzed using a panel of antibodies reactive against T cell, B cell, and myelomonocytic antigens. Only one patient was reclassified as a possible histiocytic malignancy after reevaluation. Similar immunophenotyping studies have also shown cases previously diagnosed as MH or THL express lymphoid antigens, and would now be classified as Ki-1 positive anaplastic large cell lymphoma (ALCL) or some other hematopoietic neoplasm. These results indicate true histiocytic neoplasms are extremely rare, and previous concepts concerning clinical presentation and therapeutic outcome of the entities are inaccurate. In this paper we summarize the results of multiple retrospective analyses of cases previously diagnosed as MH or THL, including our experience at University of Minnesota, to illustrate the overall rarity of these entities. The current literature on malignant histiocytic disorders is reviewed, and the clinical presentation of patients determined to have histiocytic malignancies using contemporary analytical techniques is discussed.
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PMID:Malignant histiocytosis: a reassessment of cases formerly classified as histiocytic neoplasms and review of the literature. 775 95