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)

Myelofibrosis is observed in 1/5 of the cases of C.M.L. It consists of reticulin fibers with few collagen and no osteosclerosis. It involves signs which usually indicate the extension of the myelosis to other organs and other types of cells: hepatosplenomegaly, erythroblastosis, thrombocytemia. Its prognosis is always bad. In one third of the cases, myelofibrosis develops early, and in two third it is late. Chimiotherapy is not responsible for it. These forms of C.M.L. with myelofibrosis appear as a special type of myeloproliferative disorder apart from the true C.M.L. and the true osteomyelofibrosis.
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PMID:[Myeloid leukemia with myelofibrosis (author's transl)]. 12 27

Serum angiotensin-converting enzyme in a patient with type 2 acute neuronopathic Gaucher's disease (242 nmol/min/ml) was 10.8 times higher than values for eight patients with other hereditary neurologic abnormalities (22.5 +/- 2.0) and 9.4 times higher than those for 12 patients with other diseases (25.7 +/- 2.6) (P less than 0.001). Serum lysozyme was not elevated in the patient with type 2 Gaucher's disease. These results indicate that elevated serum angiotensin-converting enzyme in an infant with neurologic involvement and hepatosplenomegaly is suggestive of the possibility of type 2 Gaucher's disease. Typical Gaucher's cells and fibrosis were observed by light and electron microscopy of the liver. An aspect hitherto unreported in Gaucher's disease or in the liver was that approximately 20% of the collagen fibrils were of the long-spacing type, with periodicity of 1,000 to 1,100 A and diameters of 900 to 1,500 A.
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PMID:Marked elevation of serum angiotension-converting enzyme and hepatic fibrosis containing long-spacing collagen fibrils in type 2 acute neuronopathic Gaucher's disease. 20 29

Costal cartilage (biopsy) from a 13-year-old boy receiving prolonged prednisone treatment (discontinuously from 2 6/12 to 13 11/12 years) for hepatosplenomegaly and lymphadenopathy has been studied and compared with costal cartilage from untreated individuals. Optical and electron microscopic studies including histochemistry have been employed. Chondrocyte degeneration characterized by lipidic material and glycogen in cells is enhanced following prednisone treatment. Acid proteoglycans are reduced in comparison to those in the untreated controls. Amianthoid (asbestoid) collagen fibers, derived from electron-dense bodies which represent remnants of degenerating chondrocytes, occur in all cartilages. A type of collagen similar to fibrous long-spacing collagen has been observed and is prominent in cartilage from the prednisone-treated individual. Evidence suggests that this type of collagen is cellular in origin and represents a transitional form of native collagen. Morphologic changes suggestive of aging are present following prolonged prednisone treatment.
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PMID:The effect of prolonged prednisone treatment on human costal cartilage. 110 31

Hepatosplenomegaly accompanied with different intestinal troubles is more or less a marked clinical manifestations in children. The histopathological and histochemical changes were studied in biopsied materials taken from the sigmoidal and rectal tissues of 49 children. These children had schistosomiasis mansoni (26), schistosomiasis and amoebiasis (4), schistosomiasis and tuberculosis (TB.) (2), amoebiasis (4), thalassemia (6), acute myeloid leukaemia (AML) (1), mucopolysaccharidosis (1) and bacillary dysentery (5). The pathological changes were erosion ulceration hyperplasia, atrophy, crypt-abscess and fibrosis (mucosa) and oedema, congestion, cellular infiltration (Lamina propria). The chemical changes were the mucin secretion, deposition of collagen and fibrin and activity of the argentaffin cells. Not all the disease agent had the same effect, but changes were marked mainly in children with S. mansoni and/or E. histolytica.
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PMID:Histopathological and histochemical studies on the sigmoidal and rectal tissues of hepatosplenic children with gastro-intestinal troubles. 143 Dec 83

We report on a 19-year-old girl with hepatosplenomegaly and possible hematological disease. We suspected Gaucher's disease on account of histological and biochemical evidence found in specimens from the liver, spleen, and bone marrow. 18 months later, pebbled skin developed on her neck and upper back. Histological examination revealed large amounts of mucous material between the collagen bundles deep in the dermis, which proved to be dermatan sulfate. The clinical and histological symptoms are characteristic for Hunter's disease.
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PMID:[Forme fruste of Hunter's disease]. 249 95

POEMS (polyneuropathy, organomegaly [hepatosplenomegaly or lymphadenopathy], endocrinopathy, M protein, and skin changes) syndrome is an uncommon plasma cell dyscrasia with diverse manifestations, including lymphadenopathy as well as those that comprise the acronym. Dermatologic changes may include hyperpigmentation, thickened skin, hypertrichosis, and papular angiomas. These changes are believed to result from humoral products of the plasma cell clone and frequently resolve with treatment of the neoplasm. We describe a man with typical POEMS syndrome with a unique skin lesion: a large, sharply circumscribed, pigmented plaque on the anterior chest wall centered over a plasmacytoma of the sternum. Results of histologic examination showed a proliferation of capillaries of varying size, enlarged fibroblasts, and increased amounts of collagen and proteoglycan from the dermis to the periosteum. Because the outer cortex of the sternum was eroded, peptide mediators of vascular and fibroblast proliferation may have diffused directly from the plasma cell lesion into the tissues of the chest wall.
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PMID:Plasma cell dyscrasia: a case of POEMS syndrome with a unique dermatologic presentation. 268 Dec 92

Clinical and morphological findings are described in 6 patients with malignant (acute) myelosclerosis/fibrosis (MMS). Haematological data are characterized by severe anaemia and thrombocytopenia, frequently accompanied by a leucopenia with an increase in myeloblasts and promyelocytes in the peripheral blood count. There is an absence of, or a minimal hepatosplenomegaly and the survival times after onset of clinical symptoms to death range from 4-12 months. The histopathology of the bone marrow shows a conspicuous proliferation of blasts (myeloblasts, promyelocytes and megakaryoblasts) in a variable amount, besides a fibrosclerosis consisting of reticulin and collagen fibrils. A comparison of MMS with ordinary myelofibrosis/osteomyelosclerosis (MF/OMS) of a chronic course implicates two important facts: (a) evolution of fibrosclerosis takes a considerable period of time for manifestation, which ranges between 20-30 months; (b) the histopathology of MMS is identical with those features observable in the rare event of a terminal stage, i.e. blastic transformation of chronic MF/OMS. Consequently MMS should be designated as an accelerated variant of MF/OMS with a rather early occurrence of a blastic crisis. The insidious onset with the dominant clinical finding of anaemia is probably responsible for the relatively late appearance of symptoms, while the progressive course prevents an overt myeloid metaplasia with a massive hepatosplenomegaly.
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PMID:Malignant (acute) myelosclerosis--a clinical and pathological study in 6 patients. 661 83

Out of 23 cases with a final diagnosis of idiopathic fever, 20 had self-limited fever with complete resolution. Comparing the cases of self-limited fever with other groups of patients with fever of unknown origin, the following differences were apparent: compared with tumoral and collagen diseases, self-limited fever occurred more frequently below age 40, the difference being significant (p less than 0.01). Chills occurred more frequently in self-limited fever than in tumoral and collagen diseases, while the incidence was similar in infectious diseases. Infectious, tumoral and collagen diseases presented with significantly greater weight loss (p less than 0.01) than self-limited fever. A greater incidence of hepatosplenomegaly was noted in self-limited fever than in infectious diseases. Hemoglobin and erythrocyte sedimentation rate (ESR) were significantly higher in self-limited fever than in the other illnesses. The NBT test was positive, with a reduction superior to 30% in the six cases in whom it was performed. In nine cases various invasive procedures were utilized: radiology, biopsy, laparoscopy (two cases), and laparotomy (one case). The data on the present series of self-limited idiopathic fever support an infectious origin of the disease because of the following: absence of an age difference with the group with a demonstrated infectious cause; fever of less than two months duration in most cases; presence of chills and less incidence of weight loss, anemia, and elevated ESR in relation to the other groups of fever of unknown origin; a positive NBT test; and spontaneous evolution to complete resolution of the disease.
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PMID:[Self-limited idiopathic fever. A study of twenty cases (author's transl)]. 721 24

This report concerns the analysis of 100 cases of fever of unknown origin, defined according to the criteria of Petersdorf and Beeson. An etiological diagnosis could be reached in 77 cases, distributed as follows: infections, 32 cases; tumors, 14 cases; collagen diseases, 13 cases; various etiologies, 18 cases; and idiopathic, 23 cases of whom 20 had selflimited fever. There were 51 male and 49 female. The incidence of infectious diseases was significantly higher below age 40 when compared with the group of collagen diseases. Recurrent fever was common (43 cases) while continuous fever was unusual (3 cases). Recurrent fever was most frequent in neoplastic diseases, the difference in relation to the other groups being statistically significant (p less than 0.05). Chills, diffuse perspiration and myalgia were more frequent in infectious diseases (p less than 0.01) than in the other groups. Collagen diseases had significantly higher (p less than 0.05) white blood cell counts than neoplastic diseases, while the latter had significantly higher (p less than 0.01) serum lactic-dehydrogenase and alpha 2 globulin levels than infectious and collagen diseases. Hepatosplenomegaly was present more frequently in neoplastic than in infectious diseases. When evaluating diagnostic procedures it was noted that invasive techniques (arteriography, biopsy, laparoscopy, laparotomy) were essential to arrive to the final diagnosis in 47 cases, while noninvasive procedures (serological and immunological tests, bacteriology, conventional radiology, clinical course, and response to therapy) were sufficient in 22 cases. In eight cases the etiology of the fever could only be determined by post-mortem examination.
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PMID:[Fever of unknown origin. A study of 100 cases (author's transl)]. 721 37

Angioimmunoblastic lymphadenopathy is a rare clinical entity, first described in 1974, characterized by asthenia, anorexia, fever, sweating, generalized lymph node enlargement, hepatosplenomegaly, rash, hypergammaglobulinemia, and often Coomb's positive hemolytic anemia. Main histopathologic findings are lymphoplasmocytic and immunoblastic proliferations, increased vascular neshwork and interstitial granular PAS positive material deposits. A lymph node excised from a woman with angioimmunoblastic lymphadenopathy was examined under electron microscopy. Results of ultrastructural study are compared to the typical histologic pattern observed under light microscopy. Analysis of the cellularity and the significance of fibrous collagen found in the interstitial PAS positive material are commented on.
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PMID:[Angioimmunoblastic lymphadenopathy. Case report with ultrastructural study (author's transl)]. 741 34


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