Malignant angioendotheliomatosis is an angiotropic intravascular lymphoma. Immunohistochemical, ultrastructural, and molecular genetics studies. 1995

C Perniciaro, and R K Winkelmann, and M S Daoud, and W P Su
Department of Dermatology, Mayo Clinic, Jacksonville, Florida 32224, USA.

Malignant angioendotheliomatosis is a rare intravascular (angiotropic) lymphoma. Patients most often present with cutaneous or central nervous system findings. We describe three patients with malignant angioendotheliomatosis involving the skin. The initial lesions in each were tender, indurated nodules on the lower extremities, resembling inflammatory panniculitis. Skin biopsies and immunohistochemical studies from all patients confirmed intravascular B-cell lymphoma. Two patients had visceral involvement, and molecular genetics studies showed clonal immunoglobulin gene rearrangement in one. Electron microscopy in this case showed increased fibrin and atypical lymphocytes within blood vessels. Malignant angioendotheliomatosis is a monoclonal intravascular lymphoma, usually of B-cell phenotype. Occlusion of small blood vessels with lymphoid cells, fibrin, and degenerating cellular debris causes the cutaneous lesions. An excisional biopsy through the depth of subcutaneous tissue may be necessary to confirm the diagnosis of malignant angioendotheliomatosis.

UI MeSH Term Description Entries
D008214 Lymphocytes White blood cells formed in the body's lymphoid tissue. The nucleus is round or ovoid with coarse, irregularly clumped chromatin while the cytoplasm is typically pale blue with azurophilic (if any) granules. Most lymphocytes can be classified as either T or B (with subpopulations of each), or NATURAL KILLER CELLS. Lymphoid Cells,Cell, Lymphoid,Cells, Lymphoid,Lymphocyte,Lymphoid Cell
D008228 Lymphoma, Non-Hodgkin Any of a group of malignant tumors of lymphoid tissue that differ from HODGKIN DISEASE, being more heterogeneous with respect to malignant cell lineage, clinical course, prognosis, and therapy. The only common feature among these tumors is the absence of giant REED-STERNBERG CELLS, a characteristic of Hodgkin's disease. Non-Hodgkin Lymphoma,Diffuse Mixed Small and Large Cell Lymphoma,Diffuse Mixed-Cell Lymphoma,Diffuse Small Cleaved-Cell Lymphoma,Diffuse Undifferentiated Lymphoma,Lymphatic Sarcoma,Lymphoma, Atypical Diffuse Small Lymphoid,Lymphoma, Diffuse,Lymphoma, Diffuse, Mixed Lymphocytic-Histiocytic,Lymphoma, High-Grade,Lymphoma, Intermediate-Grade,Lymphoma, Low-Grade,Lymphoma, Mixed,Lymphoma, Mixed Cell, Diffuse,Lymphoma, Mixed Lymphocytic-Histiocytic,Lymphoma, Mixed Small and Large Cell, Diffuse,Lymphoma, Mixed-Cell,Lymphoma, Mixed-Cell, Diffuse,Lymphoma, Non-Hodgkin's,Lymphoma, Non-Hodgkin, Familial,Lymphoma, Non-Hodgkins,Lymphoma, Nonhodgkin's,Lymphoma, Nonhodgkins,Lymphoma, Pleomorphic,Lymphoma, Small Cleaved Cell, Diffuse,Lymphoma, Small Cleaved-Cell, Diffuse,Lymphoma, Small Non-Cleaved-Cell,Lymphoma, Small Noncleaved-Cell,Lymphoma, Small and Large Cleaved-Cell, Diffuse,Lymphoma, Undifferentiated,Lymphoma, Undifferentiated, Diffuse,Lymphosarcoma,Mixed Small and Large Cell Lymphoma, Diffuse,Mixed-Cell Lymphoma,Mixed-Cell Lymphoma, Diffuse,Non-Hodgkin's Lymphoma,Reticulosarcoma,Reticulum Cell Sarcoma,Reticulum-Cell Sarcoma,Sarcoma, Lymphatic,Sarcoma, Reticulum-Cell,Small Cleaved-Cell Lymphoma, Diffuse,Small Non-Cleaved-Cell Lymphoma,Small Noncleaved-Cell Lymphoma,Undifferentiated Lymphoma,Diffuse Lymphoma,Diffuse Lymphomas,Diffuse Mixed Cell Lymphoma,Diffuse Mixed-Cell Lymphomas,Diffuse Small Cleaved Cell Lymphoma,Diffuse Undifferentiated Lymphomas,High-Grade Lymphoma,High-Grade Lymphomas,Intermediate-Grade Lymphoma,Intermediate-Grade Lymphomas,Low-Grade Lymphoma,Low-Grade Lymphomas,Lymphatic Sarcomas,Lymphocytic-Histiocytic Lymphoma, Mixed,Lymphocytic-Histiocytic Lymphomas, Mixed,Lymphoma, Diffuse Mixed-Cell,Lymphoma, Diffuse Undifferentiated,Lymphoma, High Grade,Lymphoma, Intermediate Grade,Lymphoma, Low Grade,Lymphoma, Mixed Cell,Lymphoma, Mixed Lymphocytic Histiocytic,Lymphoma, Non Hodgkin,Lymphoma, Non Hodgkin's,Lymphoma, Non Hodgkins,Lymphoma, Nonhodgkin,Lymphoma, Small Non Cleaved Cell,Lymphoma, Small Noncleaved Cell,Lymphosarcomas,Mixed Cell Lymphoma,Mixed Cell Lymphoma, Diffuse,Mixed Lymphocytic-Histiocytic Lymphoma,Mixed Lymphocytic-Histiocytic Lymphomas,Mixed Lymphoma,Mixed Lymphomas,Mixed-Cell Lymphomas,Non Hodgkin Lymphoma,Non Hodgkin's Lymphoma,Non-Cleaved-Cell Lymphoma, Small,Non-Hodgkins Lymphoma,Noncleaved-Cell Lymphoma, Small,Nonhodgkin's Lymphoma,Nonhodgkins Lymphoma,Pleomorphic Lymphoma,Pleomorphic Lymphomas,Reticulosarcomas,Reticulum Cell Sarcomas,Reticulum-Cell Sarcomas,Sarcoma, Reticulum Cell,Small Cleaved Cell Lymphoma, Diffuse,Small Non Cleaved Cell Lymphoma,Small Non-Cleaved-Cell Lymphomas,Small Noncleaved Cell Lymphoma,Small Noncleaved-Cell Lymphomas,Undifferentiated Lymphoma, Diffuse,Undifferentiated Lymphomas
D008297 Male Males
D008854 Microscopy, Electron Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen. Electron Microscopy
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D008967 Molecular Biology A discipline concerned with studying biological phenomena in terms of the chemical and physical interactions of molecules. Biochemical Genetics,Biology, Molecular,Genetics, Biochemical,Genetics, Molecular,Molecular Genetics,Biochemical Genetic,Genetic, Biochemical,Genetic, Molecular,Molecular Genetic
D010641 Phenotype The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment. Phenotypes
D001808 Blood Vessels Any of the tubular vessels conveying the blood (arteries, arterioles, capillaries, venules, and veins). Blood Vessel,Vessel, Blood,Vessels, Blood
D005260 Female Females
D005337 Fibrin A protein derived from FIBRINOGEN in the presence of THROMBIN, which forms part of the blood clot. Antithrombin I

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