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)

A case of histiocytic medullary reticulosis in which nasal involvement was predominant is reported. The patient was a 33-year-old woman with a 14-month history of unilateral nasal stuffiness. The diagnosis was established by antemortem examination of films of bone marrow aspirates and by clinical features including fever, wasting, hepatosplenomegaly, anemia, and leukopenia. The histologic examination of autopsy specimens disclosed proliferation of histiocytes, which ingested nuclear debris and closed proliferation of histiocytes, which ingested nuclear debris and erythrocytes, in the necrotic lesion of the nose, sternal bone marrow, liver, spleen, thymus, uterus, ovali, and ileum. On reviewing literature on this subject, such a case of histiocytic medullary reticulosis which predominantly involves the nose is very rare.
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PMID:Histiocytic medullary reticulosis with involvement of the nose. 97 2

A variant strain of Rauscher leukemia virus (RLV-A) obtained from a transplantable murine monomyelocytic leukemia causes a disease characterized by frank anemia, wasting, hepatosplenomegaly and erythroblastosis. The involvement of platelets in this disease are reported here. The RLV-A induced a severe thrombocytopenia (25 percent of control level) at the terminal stage of disease. This thrombocytopenia was not associated with disseminated intravascular coagulopathy since the prothrombin times were always within normal limits. The partial thromboplastin time was elevated in the terminal stages of disease and was found to be associated with factor deficiencies, possibly owing to the presence of anti-factor antibodies, in the intrinsic coagulation pathway, especially factor VIII. Further, splenectomy did not abolish the thrombocytopenia, since splenectomized, virally infected animals also developed severe thrombocytopenia (29 percent of control levels). The ensuing splenomegaly during progression of disease was not the cause of the thrombocytopenia. A physiological response to the severe thrombocytopenia was the production of larger size platelets. At terminal stages of the disease, platelet volume increased to 4.2 mu 3 (normal is 3.0 mu 3). An increase in platelet volume was also observed in splenectomized, virally infected animals. Electron microscopy indicated that these circulating platelets contained c-type viral particles. Viral infection was associated with decreased life span of circulating platelets, as measured by 75Se-methionine at mid and terminal stages of the disease. Our results suggest that direct viral infection of platelets and/or megakaryocytes with subsequent cell lysis is a possible cause of the observed thrombocytopenia observed in RLVA-induced disease and may also occur in other retrovirally-induced diseases.
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PMID:Thrombocytopenia in a retrovirally-induced murine erythroleukemia. 145 28

Parasitic infections and malnutrition coexist in many tropical and subtropical areas. Studies of Leishmania donovani and of experimentally infected Syrian hamsters have provided important insights into the complex interrelationships between malnutrition and this parasitic disease. Malnutrition, which adversely affects cell-mediated immunity, is associated with the development of visceral leishmaniasis (kala-azar) in children living in endemic areas. In turn, L. donovani can cause wasting as well as hepatosplenomegaly, fever, and anemia. Syrian hamsters infected with L. donovani develop a disease that is comparable to that of humans with kala-azar. Weight loss in infected hamsters is associated with splenic macrophage secretion of potentially catabolic cytokines as measured by the D10.G4.1 assay for interleukin-1 and the L929 cytotoxicity assay for tumor necrosis factor/cachectin. Although decreased food intake contributes to wasting in infected hamsters, studies of skeletal muscle function indicate that it is not the sole factor. Leishmania donovani-infected hamsters have also been used to study drugs with the potential to prevent or reverse cachexia.
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PMID:Visceral leishmaniasis: a model for infection-induced cachexia. 163 76

Nine black children aged between 3 months and 30 months of age, with human immunodeficiency virus type I (HIV-I) infection are described to draw the attention of health professionals in southern Africa to special clinical characteristics useful for recognising this problem, which has many shared features with common diseases of infancy and childhood in the Third World. The main presenting complaints were chronic cough and persistent diarrhoea and vomiting. These children frequently had diarrhoea (8 of 9 patients), mucocutaneous candidiasis (8), pneumonia (7), hepatosplenomegaly (9), significant lymphadenopathy (5) and wasting (5). All were infected by common bacteria, such as Gram-negative organisms, Mycobacterium tuberculosis and Campylobacter jejuni, or by opportunistic infections such as Candida or cytomegalovirus (CMV), or by both bacterial and opportunistic organisms. A raised total serum globulin level, anaemia, lymphopenia and a cerebrospinal fluid (CSF) pleocytosis were frequent findings. Incomplete data on parental HIV status suggest perinatal transmission. Three of the children were HIV-antigen positive. The diagnosis of full-blown acquired immunodeficiency syndrome (AIDS), using the stringent Centers for Disease Control criteria, is difficult in our situation because of limited diagnostic resources; however, using these criteria, and the clinical case definition for AIDS recommended by World Health Organisation, it is thought that probably 4 of these children could be considered as having AIDS.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Some early observations on HIV infection in children at King Edward VIII Hospital, Durban. 223 85

Three patients with peripheral T-cell lymphoma presenting with pyrexia, wasting, hepatosplenomegaly and pancytopenia in the absence of myelophthisic lymphomatous involvement are reported. Early in the course of the disease when there was no significant lymphadenopathy, these cases created enormous diagnostic confusion. Although the clinical features were suggestive of malignant histiocytosis (MH), marrow findings showed phagocytic histiocytes which did not appear atypical, and the criteria for diagnosis of MH could not be satisfied. Lymph node enlargement was detected only after 14, 5, and 8 weeks from the onset of symptoms, and the diagnosis of T-lymphoma was then made on lymph node biopsies. Treatment with multiple agent chemotherapy was attempted. Two patients died 3 days and 11 weeks after treatment was started and the third was lost to follow-up. In contrast with most of the cases reported in the literature, our cases show that a reactive hemophagocytic syndrome can be an early and prominent manifestation of an underlying T-cell lymphoma. Differentiation from other causes of hemophagocytic syndrome can be difficult and lack of histological proof of malignancy in the initial stage often delays definitive diagnosis and treatment.
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PMID:Peripheral T-cell lymphoma presenting as hemophagocytic syndrome. 278 35

This is a report of a long term prospective study of 13 seriously ill patients with Budd-Chiari syndrome as a result of occlusion of the hepatic veins who were treated by side to side portacaval shunt from four to 78 weeks after the onset of symptoms and who were under observation for three to 16 years. The patient population was young, ranging in age from 19 to 45 years; seven were men and six were women. The presumed cause was the use of oral contraceptives in three, polycythemia rubra vera in two, Behcet disease in one patient and unknown in seven patients. All of the 13 patients had abdominal pain, marked ascites, hepatosplenomegaly, wasting and disturbed liver function. Diagnosis was based on the symptoms and signs: angiographic demonstration of hepatic vein occlusion and a patent inferior vena cava; pressure measurements that showed an inferior vena caval pressure that was normal or within the usual range for patients with massive ascites and an elevated wedged hepatic vein pressure that was much higher than the inferior vena caval pressure, and the results of biopsy of the liver showing centrilobular congestion and necrosis. Side to side portacaval shunt was very effective in decompressing the liver, reducing the mean corrected portal pressure from 240 millimeters of saline solution before to 7 millimeters of saline solution after the shunt. Operative survival rate was 92 per cent, and the long term survival rate for three to 16 years is 85 per cent. All of the survivors are free of ascites without requiring diuretic therapy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Long term results of treatment of Budd-Chiari syndrome by side to side portacaval shunt. 290 30

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

The clinical course of 10 cases where marrow aspirate showed features of haemophagocytosis was reviewed. Eight of these had a fulminant clinical course characterized by high fever, constitutional symptoms, wasting, hepatosplenomegaly with liver dysfunction, sometimes lymphadenopathy, progressive pancytopenia and coagulopathy, like that described as 'malignant histiocytosis' in the past. The remaining 2 cases did not have this classical clinical syndrome. Among the former 8 cases, 4 of them had high-grade lymphoma, 3 of whom were confirmed to be peripheral T cell lymphoma. Three of the remaining 4 had suspicious lymphomatous infiltrate on marrow trephine. In every case an extensive search for viral etiology by serology was negative. The 2 cases which did not have fulminant clinical feature were found to have lymphoma of the diffuse large cell and Ki-1 anaplastic type, respectively. A review of the literature reveal that most cases with haemophagocytic syndrome have a fulminant clinical course and are peripheral T cell lymphoma, which generally has a poor prognosis. In our study, the 8 cases with the classical haemophagocytic syndrome had a median survival of 24 days and a long-term survival of 37.5% at 28 months. Prompt initiation of chemotherapy is a life-saving measure and the only chance of achieving a long-term survival in patients with haemophagocytic syndrome if the underlying lymphoma can be diagnosed early.
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PMID:Haemophagocytosis in bone marrow aspirate--a review of the clinical course of 10 cases. 935 53

A retrospective study of all children admitted with the diagnosis of acquired immunodeficiency syndrome (AIDS) at Jos University Teaching Hospital (JUTH) between August 1995 and October 1996 was carried out. Forty three (1.5% out of a total of 2793 children were diagnosed with HIV infection during the study period. However, only the records of 23 out. Of the 43 positive cases were available for analysis. Of the 23 cases whose records were available, 8 presented in 1995, while the remaining 15 presented between January and October 1996. The ages of the children ranged between 1 and 15 years (Mean 3.0 +/- 4.1 Years). There were 12 males and 11 females (M:F = 1:1). Sixteen (69.6 percent) out of the 23 patients were aged between 1 month and 2 years. Sixteen (69.6%) of the 23 patients acquired the infection vertically, 2 (8.9%) acquired the infection through blood transfusion, 1 (4.3%) from sexual abuse, while in 4 (17.4%) the source of infection could not be established due to inadequate data. Majority of the children presented with weight loss, chronic diarrhoea and fever, while the common findings included wasting, oralthrush, pallor, hepatosplenomegaly and lymphadenopathy. Six (26.1 percent) out of the 23 children died, 8 (34.8 percent) were discharged against medical advice and have not been seen since, 9 (39.1%) improved and were discharged to out-patient clinic followup, but all except 2 of these have been lost to follow-up. It is concluded that AIDS is increasingly becoming a major cause of childhood morbidity and mortality in our environment. All children in our environment who present with features of malnutrition should be screened for AIDS. Campaigns aimed at preventing vertical (maternal-child) transmission, including health education of young men and women on the risk of unprotected sex must be vigorously pursued and sustained.
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PMID:Paediatric AIDS in Jos, Nigeria. 992 Oct 95

A case of malignant histiocytosis (MH) in a 12-year-old male child is described. Fever and wasting were the most prominent symptoms. Bilateral cervical and axillary lymphadenopathy was present along with hepatosplenomegaly. The haematological, cytological and histopathological features are described. The diagnostic dilemma presented during the diagnosis of this case is also discussed. Diagn. Cytopathol. 1999;21:359-361.
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PMID:Malignant histiocytosis in childhood: A case report. 1052 86


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