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)

This report details an unusual case of Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disorder (PTLD) involving the epicardial coronary arteries and myocardial arterioles of an allograft heart, morphologically mimicking vasculitis, a finding associated with acute rejection, cardiac allograft vasculopathy (CAV), infection and infarction. Recognition of this novel PTLD pattern as a potential imitator of acute rejection in particular is important because reduction of immunosuppression (to treat PTLD) is antithetical to the usual therapy for rejection.
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PMID:An unusual presentation of post-transplant lymphoproliferative disorder mimicking vasculitis in heart transplantation. 1897 Nov

A 50-year-old man presented with an asymptomatic, 1.5 x 1.5 cm, dark-brown noduloplaque with a rubbery consistency (Fig. 1) on the lateral aspect of the left lower leg of uncertain duration. His general condition was healthy, and he did not recall any trauma or insect bite at this site. No similar skin lesions were found elsewhere and no lymphadenopathy was observed. The lesion revealed a nonencapsulated, but well-circumscribed, deep dermal nodule with several lymphoid aggregates and germinal center-like structures within the tumor and also at the periphery, when examined microscopically at scanning power (Fig. 2a). The epidermis showed no remarkable changes, except for basal hyperpigmentation. At higher power, a mixed inflammatory infiltrate composed of histiocytes, foamy histiocytes (Fig. 2b), lymphocytes, and abundant plasma cells (Fig. 2c) with Russell bodies was revealed. The stroma contained mainly hyalinized and sclerotic collagen fibers (Fig. 2d). Prominent venules were noted, especially in the sclerotic areas, and some were surrounded by dense collagen fibers. No vasculitis or emperipolesis was found. No foreign materials were observed by polarization microscopy, and no organisms could be identified by periodic acid-Schiff (PAS), Grocott methenamine silver (GMS), Giemsa, Gram, acid-fast, or fite stains. The results of testing for infection by Epstein-Barr virus (EBV) (latent membrane protein 1, LMP-1) were negative. No spindle cells were found in the lesion. Immunohistochemical studies demonstrated mature plasma cells stained with CD138, and polyclonality was confirmed by the expression of both kappa and lambda light chains. The germinal center-like lymphoid aggregates were found to be B cells, which reacted positively with CD20. Scarce S100-positive cells and even rarer CD1a-positive cells were detected. Test results for smooth muscle actin (SMA) and anaplastic lymphoma kinase (ALK) were negative. Abundant CD68+ macrophages were observed within the lesion (Fig. 3a), and about 50-75% of the inflammatory cells were found to express cyclooxygenase-2 (COX-2) (Fig. 3b). The patient's condition was diagnosed as cutaneous plasma cell granuloma (CPCG). One year after excision, no evidence of recurrence was observed.
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PMID:Cutaneous plasma cell granuloma: report of a case with novel histologic and immunohistochemical findings. 1933 29

X-linked lymphoproliferative disease (XLP) is an immunodeficiency caused by defects in the adaptor molecule SAP. The manifestations of XLP generally occur following Epstein-Barr virus (EBV) infection and include fulminant mononucleosis, hypogammaglobulinemia and lymphoma. In this report, we describe two unrelated patients with fatal T-cell-mediated central nervous system vasculitis for whom repeated serologic and molecular testing for EBV was negative. In both patients, clonal T-cell populations were observed, but neither demonstrated evidence of lymphoma. Thus, loss of SAP function can lead to dysregulated immune responses characterized by the uncontrolled expansion and activation of T cells independent of EBV infection.
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PMID:Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection. 1962 58

Hydroa vacciniforme (HV) is a rare, blistering, childhood photodermatosis that heals with smallpox-like scars, is associated with latent Epstein-Barr virus (EBV) infection, and spontaneously resolves by early adulthood. Cytotoxic T cells are suspected to mediate the histologic hallmark of HV-dense, perivascular, lymphocytic infiltrates and reticular degeneration and necrosis of the epidermis. We report a case of 13-year-old white girl with EBV-associated HV, whose lesional skin harbored a predominate CD68, CD123 infiltrate of plasmacytoid monocytes (PMs). Other significant pathologic attributes included abundant non-neutrophilic nuclear debris, necrosis of adnexal structures, lymphocytic vasculitis, and clusters of CD30 cells. PMs produce large amounts of type I interferon during viral infection, which induces apoptosis of some cell types and promotes the survival of others. This antiviral response can explain these aforementioned pathologic findings representing wide spread cell death and lymphoid proliferation, as well as eventual resolution of HV with time, via elimination of latent EBV-infected cells. The destruction of adnexal structures can account for the depressed circinate scars characteristic of HV. The validity of this proposed mechanism must be evaluated by first examining the prevalence and number of PMs in future case series of HV.
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PMID:Plasmacytoid (CD68+CD123+) monocytes may play a crucial role in the pathogenesis of hydroa vacciniforme: a case report. 1973 83

Lymphomatoid granulomatosis (LYG) is a rare angiocentric and angiodestructive lymphoproliferative disease characterized by Epstein-Barr virus-positive B cells admixed with reactive T cells. LYG most commonly affects the lung but can also involve other extrapulmonary sites. Pulmonary LYG usually presents as multiple pulmonary nodules with rapid progression and excavation. It can mimic various infectious diseases, vasculitis or metastatic malignancy and is difficult to be diagnosed clinically. Standard treatment for LYG has not yet been established. Despite combination chemotherapy, the overall prognosis is poor. Recently, anti-CD20 monoclonal antibody, rituximab, has been used to treat LYG. We report the case of a 70-year-old male patient with pulmonary LYG, who showed rapid remission of the disease after combination chemotherapy with rituximab.
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PMID:A case of pulmonary lymphomatoid granulomatosis successfully treated by combination chemotherapy with rituximab. 1975 54

In this study we assess the presence of antibodies against infectious agents as well as for a variety of autoantibodies in an attempt to establish associations between various vasculitides and infections in order to shed light on the etiopathogenesis of these diseases and perhaps implicate a potential cure. Sera from patients with Wegener's granulomatosis (WG), polyarteritis nodosa, microscopic polyangiitis, Churg Strauss, and giant cell arteritis were compared to healthy control sera. Serum samples were assessed, using the Bio-Rad BioPlex 2200, for the presence of Toxoplama gondii, cytomegalovirus (CMV), Epstein-Barr virus (EBV), Treponema pallidum, and Saccharomyces cerevisiae. Hepatitis B virus (HBV), hepatitis C virus (HCV), and anti-Helicobacter pylori antibodies were assessed by ELISA. In addition, sera were tested for a panel of antibodies associated with thrombophilia as well as various autoantibodies. The prevalence of antibodies toward HCV and H. pylori was significantly higher among patients with WG. IgG antibodies toward T. gondii and IgM antibodies toward CMV were significantly more common among WG patients than among controls. WG patients exhibited more antibodies toward EBV viral capsid antigen IgG and EBV early antigen IgG compared to sera from healthy controls. In WG, positive associations were disclosed between CMV IgG antibodies and the presence of gastrointestinal manifestations and renal involvement, and there was a higher Birmingham vasculitis activity score in association with elevated titers of EBV viral capsid antigen IgG antibodies. Otorhinolaryngeal manifestations were more common in those with positive IgG antibodies for EBV early antigen. Our results unveil novel associations between WG and various infectious agents, including HCV, H. pylori, T. gondii, CMV, and EBV. In addition to putative roles in initiation and exacerbation of the vasculitic process, it seems that these infectious agents also modulate the clinical phenotype of the disease.
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PMID:Infectious serologies and autoantibodies in Wegener's granulomatosis and other vasculitides: novel associations disclosed using the Rad BioPlex 2200. 1975 11

We describe a 66-year-old woman with Epstein-Barr virus-associated lymphoproliferative disorder with lung and gastric tumors. We identified two lung tumors measuring 13 and 20 mm in diameter that consisted of CD30-, CD15-, and CD20-positive Hodgkin- and Reed-Sternberg-like cells and heterogeneous cellular infiltrates in a pronounced nodular pattern, with necrosis and vasculitis, diagnosed as nodular sclerosis classical Hodgkin lymphoma. A gastric tumor showed low-grade extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type. Neoplastic cells in all tumors expressed Epstein-Barr virus-encoded RNA based on in situ hybridization. The present case is a rare composite lymphoma arising from different extranodal organs, associated with EBV infection. Her medical history included gamma-knife therapy for clinical diagnosis with a suspicion of cerebral lymphoma.
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PMID:Coexistence of primary pulmonary Hodgkin lymphoma and gastric MALT lymphoma associated with Epstein-Barr virus infection: a case report. 2059 74

We report a case of an adult patient who presented a febrile coma linked to Epstein-Barr virus (EBV) encephalitis. EBV polymerase chain reaction (PCR) was positive in cerebrospinal fluid (CSF) and blood serology and PCR in blood was consistent with an EBV reactivation. First cerebral magnetic resonance imaging (MRI) at day 1 was normal but a second MRI at day 13 showed anomaly compatible with a hemorrhagic leukoencephalitis. Treatment consists of intravenous corticotherapy and aciclovir during 21 days. Evolution was favourable with complete neurologic recuperation and no intercurrent lymphoma or vasculitis in 6 months follow-up.
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PMID:Hemorrhagic leukoencephalitis linked to Epstein-Barr virus in an adult patient. 2188 58

Acute hemorrhagic edema (AHE) is an uncommon self-limited disorder affecting young children triggered by infection, drugs, or immunization. A 2-year-old boy was observed due to sudden onset of painful and edematous purpuric papular and plaque lesions of the face and upper extremities that started 2 weeks after H1N1 immunization. The patient also developed exuberant edema on the face and dorsum of the hands. Complete blood count, biochemistry, and urinalysis results were normal. Histopathological examination revealed perivascular and periadnexial lymphocytic infiltrate with neutrophils and eosinophils, and leukocytoclastic vasculitis. Blood PCR technique was negative to several viruses, namely adenovirus, cytomegalovirus, Epstein Barr, enterovirus, HHV6, parvovirus B19, and H1N1. Symptomatic treatment and parents reassurance was promptly provided. However, new lesions continued to develop and in this setting systemic corticosteroid was prescribed. Complete clinical resolution was achieved within 2 weeks and no relapse was observed. The temporal relationship with H1N1 immunization, absence of previous drug intake, as well as exclusion of viral infections led the authors to propose that H1N1 vaccine was the predisposing factor in AHE development in our patient. To our best knowledge, this is the first reported association between AHE and H1N1 immunization.
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PMID:Acute hemorrhagic edema of childhood after H1N1 immunization. 2112 77

We report an 18-year old female patient with systemic lupus erythematosus (SLE), who developed fever, pancytopenia, abdominal pain, and watery diarrhea. Computed tomography (CT) and bone marrow aspirate revealed lupus mesenteric vasculitis (LMV) and hemophagocytic syndrome (HPS). Serologic tests for Epstein-Barr virus (EBV) indicated its reactivation. This case demonstrates that HPS and concomitant LMV associated with viral reactivation can occur as clinical manifestations of SLE flare.
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PMID:Simultaneous presentation of hemophagocytic syndrome and mesenteric vasculitis in a patient with systemic lupus erythematosus. 2122 76


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