Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It has been estimated that immunocytomas comprise roughly 2% of all cutaneous lymphomas. We studied five patients with primary cutaneous immunocytomas who presented with cutaneous nodules or plaques. Many of the infiltrates were "top-heavy" and polymorphous with admixed eosinophils, macrophages, lymphoid follicles, and non-neoplastic lymphocytes. Other potentially confusing findings were one case each of spongiotic dermatitis and leukocytoclastic vasculitis. The neoplastic cells were often situated at the peripheries of nodules and ranged from those with nuclei that resembled small lymphocytes to others that resembled immunoblasts. Most had eccentrically placed nuclei and fan-shaped cytoplasm. Monotypic kappa-light chain was found in all five cases, accompanied by gamma-heavy chain in two cases, and mu-heavy chain in one. In situ hybridization detected only kappa-mRNA in the four cases that yielded technically satisfactory results. The neoplastic cells did not express the B-cell antigen CD20; T-cells formed the centers of many nodules. Inappropriate staining for CD43 was evident in the neoplastic cells of one case. Because of reports of immunocytomas complicating acrodermatitis chronica atrophicans, we stained sections with an antiserum to Borrelia burgdorferi, which did not detect that organism. In situ hybridization did not detect EBER-1 RNA of the Epstein-Barr virus, which can be present in immunocytomas in immunocompromised patients. One patient died of disease after failing chemotherapy; another is alive with disseminated disease, and three are in remission following excision of lesions alone in two patients and chemotherapy in one patient who had relapsed following both excision and radiation therapy.
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PMID:Primary cutaneous immunocytoma. A B-cell lymphoma that can easily be mistaken for cutaneous lymphoid hyperplasia. 809 99

Lymphoproliferative disorders of granular lymphocytes (LDGL) represent a family of diseases characterized by persistent granular lymphocytosis with variable prognosis. The Epstein-Barr virus (EBV) has been occasionally linked with the development of LDGL. However, cutaneous manifestations of LDGL have rarely been reported. One patient had cutaneous vasculitis for 10 years before a definite diagnosis of LDGL was made. Chronic EBV infection was documented serologically and EBV DNA was detected in the peripheral blood lymphocytes. EBV RNA was detected in the nuclei of infiltrating lymphoid cells expressing CD43 in a skin biopsy specimen. A cytogenetic study showed clonal chromosomal abnormalities. This is the first report of EBV-associated LDGL of natural killer cells with cutaneous manifestations.
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PMID:Epstein-Barr virus-associated lymphoproliferative disorder of granular lymphocytes presenting initially as cutaneous vasculitis. 829 94

Chronic active Epstein-Barr virus infection (CAEBV) is a nonfamilial syndrome that shows a specific immunodeficiency for the Epstein-Barr virus (EBV). Prolonged fever, hepatosplenomegaly, lymphadenopathy, and liver dysfunction were seen in CAEBV, but cardiac complications are rare. An autopsy case of CAEBV with giant coronary aneurysms and aortic aneurysms is reported. The patient was a 5-year-old Japanese girl. At autopsy, the heart weighed 110 g, and bilateral coronary aneurysms were found. Microscopic studies revealed lymphoid vasculitis of coronary arteries, coronary venules, and aortic arteries. Immunohistochemically, infiltrating small lymphocytes were positive for CD3, CD45RO(UCHL-1), CD43(DF-T1). The presence of EBV in most of these T lymphocytes was proven by in situ hybridization using an EBV-encoded RNA-1 (EBER1) probe. To the best of the author's knowledge, pathology of aneurysms caused by lymphoid vasculitis in CAEBV has not been reported until now.
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PMID:Chronic active Epstein-Barr virus infection with giant coronary aneurysms. 865 48