Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.17 (lysozyme)
 21,489 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neoplastic angioendotheliomatosis (NAE) is a rare entity characterized by multifocal, intravascular proliferation of large pleomorphic cells within small vessels of most organs, with a particular affinity for the central nervous system. Clinically, patients with NAE present with focal neurologic signs and a progressive decline in mental status, followed by death in a few months. The histogenesis of NAE is controversial but has been previously thought to represent a malignant proliferation of endothelial cells. Three autopsy cases with clinical and histologic features of NAE were investigated by electron microscopic, standard histochemical, and immunohistochemical technics that included the use of three panleukocyte monoclonal antibodies (PLA), the endothelial-cell-specific reagents, FVIII-RAG anti-sera and Ulex europaeus agglutinin (UEA), and muramidase. The NAE cells in all three cases were stained positively by the PLA, whereas the adjacent endothelial cells and not the NAE cells were stained by FVIII-RAG and UEA. Muramidase by immunoperoxidase technic and nonspecific esterase (chloracetate) were not present in NAE cells. These results indicate that NAE is a leukocyte-derived neoplasm and not a malignant endothelial cell neoplasm. Based on these findings and on a review of the literature, it is proposed that NAE represents intravascular malignant lymphomatosis (IML). IML appears to be a primary manifestation and/or a major secondary form of disseminated malignant lymphoma. This would explain the spectrum of findings in previously reported cases.
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PMID:Neoplastic angioendotheliomatosis: immunopathologic and morphologic evidence for intravascular malignant lymphomatosis. 351 72

Two patients with systemic angioendotheliomatosis had prominent constitutional symptoms such as fever, loss of weight, and general weakness, and had multiple organ dysfunctions, including bizarre neurologic findings and dementia. Severe anemia that required frequent blood transfusions also was present. One patient developed severe hemolysis and hypersplenism that required splenectomy for relief; the other patient had intravascular hemolysis and autoimmune hemolytic anemia, which were treated unsuccessfully with conservative measures. In both cases, postmortem examination showed many large, noncohesive malignant cells within the lumen of the blood vessels in many of the organs. There was no infiltration or replacement of the normal tissues by the tumor cells. Histochemical studies showed that the tumor cells were pyroninophilic but did not have cytoplasmic immunoglobulins or activity of chloroacetate esterase and muramidase. The cells showed factor VIII antigen in their cytoplasm. Systemic angioendotheliomatosis may represent a true neoplastic process of the vascular endothelial cells.
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PMID:Systemic angioendotheliomatosis presenting with hemolytic anemia. 619 2

Clinico-pathological data in a typical case are compared with those of 54 published reports of systemic angioendotheliomatosis. In this rare disease the lesions, characterized by clusters of abnormal cells in the small vessels, usually have multivisceral localizations, with chiefly cutaneous and encephalic manifestations and a rapidly fatal course. However cases are known with lesions restricted to the skin and a protracted if not regressive course; about half of these cases are associated with an endocarditis. In the case reported, the results of ultrastructural and histo-immunological studies (f. VIII, lysozyme, anti-protease, Ig) are in favour of the endothelial origin of the abnormal cells, a now widely accepted hypothesis.
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PMID:[Malignant systemic angioendotheliomatosis. Apropos of a case with an ultrastructural and histo-immunologic study]. 636 64