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)

Malignant histiocytosis is a rare systemic disorder, characterized by progressive, invasive proliferation of atypical histiocytes. It is synonymous with the disease entity "histiocytic medullary reticulosis" initially described by Scott and Robb-Smith (Lancet 1939;194-8). To date, there have been slightly more than 100 cases reported in the literature. Although the radiographic features of malignant histiocytosis have been reported, to our knowledge there has not been any computed tomography (CT) description of the findings of this disease entity. The purpose of this report is to describe the serial CT findings of an unusual case of malignant histiocytosis in a patient with sickle cell anemia, presenting with weight loss and hepatosplenomegaly.
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PMID:Malignant histiocytosis in a patient with sickle cell anemia: CT findings. 688 45

Malignant histiocytosis (MH) is a rare, malignant neoplasm with protein manifestations, including fever, lymphadenopathy, hepatosplenomegaly, pancytopenia, and, infrequently, cutaneous lesions. A patient had initial manifestations that included fever and skin lesions. Skin lesions began on distal areas of the extremities and spread to involve proximal areas of the extremities and the abdomen. Erythematous maculopapular lesions, nodules, and plaques were present in various stages of development, which evolved into ulcerated plaques that exuded a serosanguineous discharge. Spontaneous healing of lesions occurred, leaving hyperpigmented, atrophic scars. Histologically, the deep dermis and subcutaneous tissues contained a diffuse infiltrate of histiocytes, lymphocytes, and mononuclear cells. Erythrophagocytosis by histiocytes was present in the skin biopsy material. Thirteen percent of 320 cases in the literature had skin involvement. This report and review of the literature indicate that, in selected cases, skin biopsy may contribute to the diagnosis of MH.
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PMID:Cutaneous involvement in malignant histiocytosis. Case report and review of the literature. 722 56

Malignant Histiocytosis (MH) is an uncommon disorder characterised by systemic tissue infiltration of atypical histiocytes and its precursors. The clinical features included fever with weight loss, hepatosplenomegaly and pancytopenia. The antemortem pathology of the lymph node and bone marrow were non-specific and non-diagnostic. However, at autopsy, there were florid pathological changes of malignant histiocytosis. This case illustrates the difficulty of making a definite antemortem diagnosis of this rare malignant condition and the terminology, aetiology and pathological features are discussed.
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PMID:Malignant histiocytosis --- a case report. 733 4

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

An 18-year-old man was admitted to our hospital because of 39 degrees C fever for over one month, marked hepatosplenomegaly, and pancytopenia. Malignant histiocytosis, malignant lymphoma, or hemophagocytic syndrome were ruled out by bone marrow aspiration and liver biopsy. A diagnosis of chronic EB virus infection was made according to his characteristic clinical features, abnormally high titiers of anti-EBV antibodies (VCA-IgG x 2560, EA-IgG x 1280), and the detection of EBV genome in the peripheral blood mononuclear cells by polymerase chain reaction. He also manifested granular lymphocyte proliferative disorder (GLPD). The phenotype of the proliferating granular lymphocytes was CD2 (+), CD3 (-), CD56 (+), and IL-2R beta (+), showing the NK lineage of these cells. Chromosomal abnormality of the cells cultured for a short time with IL-2 and a monoclonal junctional DNA structure of EB virus terminal repeat analyzed by the Southern blotting provided definitive evidence for the monoclonal expansion of the granular lymphocytes. These findings indicate a causative role of EV virus in NK-GLPD or NK-leukemia.
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PMID:[Chronic active EB virus infection accompanied by monoclonal proliferation of granular lymphocytes]. 778 25

Malignant histiocytosis (MH)-like B-cell lymphoma (BCL) is a neoplastic proliferation of large B cells clinically characterized by fever, hepatosplenomegaly, haemophagocytosis and abnormal laboratory data, without lymphadenopathy or skin lesions. Interestingly, most cases have been reported in Asian patients, and it is unclear whether MH-like BCL is biologically distinct from conventional large B-cell lymphomas. We report five Japanese patients with MH-like BCL. Biopsied specimens of bone marrow, liver and/or spleen showed infiltration of neoplastic B cells accompanied by haemophagocytosing histiocytes. Lymphoma cells were positive for CD19, CD20 and HLA-DR surface antigens, and negative for CD5 and CD10. In four cases elevated serum levels of interleukin (IL)-6 and the soluble IL-2 receptor isoform were noted, but not IL-1beta, IL-2 or tumour necrosis factor-alpha. Autopsies of two cases were pathologically diagnosed as intravascular lymphomatosis (IVL). Based on these observations, the current and nine previous cases reported as MH-like BCL in Japan were re-evaluated. They appear to form a peculiar variant of IVL, characterized by bone marrow involvement at presentation, haemophagocytic syndrome, and a rapidly aggressive clinical course, but rarely neurological complications or skin lesions. This variant may merit separate consideration because of the problems posed in the initial diagnosis and therapeutic approaches.
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PMID:Malignant histiocytosis-like B-cell lymphoma, a distinct pathologic variant of intravascular lymphomatosis: a report of five cases and review of the literature. 940 Oct 80

Malignant histiocytosis-like B-cell lymphoma (MH-like BCL) is characterized clinically by hemophagocytic syndrome (HPS), bone marrow involvement at presentation, and an aggressive clinical course. Yet, it remains an ill-defined disease entity. We recently described five cases of MH-like BCL and suggested that these may be regarded as a peculiar variant of intravascular lymphomatosis (IVL), based on clinical and pathological observation. Interestingly, this type of lymphoma has been reported exclusively among Asians, with few reports from Western countries, and therefore we propose that this variant be noted as an Asian variant of IVL(AIVL). To evaluate the incidence of this variant, we performed a literature search for English or Japanese studies dealing with MH-like BCL or B-cell IVL associated with HPS (IVL with HPS). We found 18 reports of MH-like BCL and 16 reports of IVL with HPS and re-evaluated the clinicopathologic aspects of each study with respect to AIVL. Although several differences were noted, such as gender, presence of respiratory changes, bone marrow invasion and disseminated intravascular coagulopathy, there were also overlapping profiles and they appeared to be consistent with the AIVL single disease entity. Of particular note was the absence of peripheral lymphadenopathy and tumor formation which posed diagnostic problems in the initial phase of the presentations. Thus, based on analysis of the clinical findings of these 34 cases, the diagnostic criteria for AIVL consists primarily of cytopenia (anemia and/or thrombocytopenia), hepatosplenomegaly, absence of peripheral lymphadenopathy and tumor formation, and erythrocyte-hemophagocytosis along with intravascular proliferation of the neoplastic B cells. The pathologic findings of the 19 autopsied cases which fulfilled the criteria are also summarized, and they reveal morphologic, immunologic, and genotypic features of typical IVL.
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PMID:An Asian variant of intravascular lymphomatosis: an updated review of malignant histiocytosis-like B-cell lymphoma. 1034 74


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