Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Vasculitic syndromes associated with infection with the human immunodeficiency virus (HIV) would appear to represent a microcosm of the vasculitic spectrum. Reported cases have included polyarteritis nodosa-like illnesses, hypersensitivity vasculitis, lymphomatoid granulomatosis, primary angiitis of the central nervous system, and a number of miscellaneous disorders. The pathogenesis of these conditions is unclear, but some appear to be mediated in part by the HIV itself. Therapeutically, little clinical data exist to guide clinicians in the management of such patients, but aggressive approaches combining immunosuppressive therapy with assertive antimicrobial prophylaxis may be warranted.
 ...
PMID:Vasculitis and infection with the human immunodeficiency virus. 204 83

We describe cases of severe odynophagia, extensive oral ulcerations, and bowel perforation in patients with human immunodeficiency virus infection that were caused by lymphomatoid granulomatosis. Such presentations in human immunodeficiency virus-infected individuals are usually ascribed to other causes and may be incorrectly treated on an empiric basis. In addition, deep tissue specimens obtained at the margin of ulcerative lesions are often necessary for definitive diagnosis. We review our limited treatment experience with zidovudine, interferon alfa, and H2 blockers in our patients. Based on the markedly increased frequency in which lymphomatoid granulomatosis is being diagnosed at our institution in the post-human immunodeficiency virus era, we postulated an association between these two entities.
 ...
PMID:Lymphomatoid granulomatosis presenting as ulcerodestructive gastrointestinal tract lesions in patients with human immunodeficiency virus infection. A new association. 224 77

Fourteen cases of vasculitis associated with human immunodeficiency virus infection have thus far been described. Five of these cases may be classified as angiocentric immunoproliferative disorders, including benign lymphocytic angiitis, lymphomatoid granulomatosis, and angiocentric lymphoma. We report a case of benign lymphocytic angiitis of T cell lineage. Extensive studies found no evidence of viral antigens in the inflammatory infiltrates, and immunologic evaluation of the pathologic lesions revealed the infiltrating cells to be predominantly CD3+, CD8+, CD4-. A significant number of these lymphocytes demonstrated a deletion of T cell antigen receptor determinants. We believe that in certain cases of human immunodeficiency virus disease, there occurs a spectrum of lymphoproliferative disorders with angiocentric features that lead to the clinical picture of systemic necrotizing vasculitis. Clinicians should be aware of this association.
 ...
PMID:Systemic vasculitis in association with human immunodeficiency virus infection. 265 5

While primary and secondary malignant lymphomas have been well-documented in the CNS of patients with the acquired immunodeficiency syndrome (AIDS), only one case of lymphomatoid granulomatosis (LG) involving the CNS has been reported. We present three AIDS patients with multiple grossly evident foci of necrosis in the cerebral hemispheres which, on histologic evaluation, were seen to contain angiocentric mixed chronic inflammatory infiltrates with atypical mononuclear cells, luminal thrombosis, and infarction, which is typical of LG. LG was also identified in sections of the lung in one case. Lymphoma was found in other regions of the brain in two cases, suggesting the evolution of LG into cerebral lymphoma. In addition, widespread perivascular multinucleate syncytial giant cells, associated with human immunodeficiency virus (HIV) infection of the CNS, were identified in all patients. The features of LG, its relationship to lymphoma, and the possible etiologic role of an immunodeficiency state or the HIV virus in the pathogenesis of LG are discussed.
 ...
PMID:Lymphomatoid granulomatosis and malignant lymphoma of the central nervous system in the acquired immunodeficiency syndrome. 270 27

Organized lymphoid aggregates are normally found within the walls of bronchi in many species and may occur, under conditions of disease, in humans. This bronchus-associated lymphoid tissue (BALT) can be viewed as an organizing principle to explain the behavior and distribution of many pulmonary lymphoid proliferations, both hyperplastic and neoplastic. The extent of lymphoid hyperplasia can vary, from multifocal proliferations that arise in and remain in the airway walls (follicular hyperplasia of BALT) to those that form a solitary mass or nodule (nodular lymphoid hyperplasia of BALT or "pseudolymphoma") to multifocal or diffuse lymphoid hyperplasia of BALT ("lymphoid interstitial pneumonitis"). It seems likely that more than one cause accounts for these proliferations. Of interest is the hypothesis that many examples of diffuse lymphoid hyperplasia associated with the acquired immune deficiency syndrome may have a viral cause, possibly human immunodeficiency virus or, in some cases, Epstein-Barr virus (EBV). Most pulmonary lymphomas are low-grade B cell lymphomas. They exhibit histological diversity in any given case, characterized by small lymphocytes with irregular nuclei and pale cytoplasm (so-called "centrocytelike" cells), scattered immunoblasts, and lymphoplasmacytic or plasma cells. Reactive germinal centers are frequently present, and this, along with the benign clinical behavior of these tumors, may cause diagnostic confusion with reactive lesions. Both the histological appearance and the clinical behavior (in particular, the tendency to recur in extranodal sites) of these low-grade lymphomas can be explained on the basis of origin in BALT. T cell lymphoproliferative processes can occur in the lung but are rare. Lymphomatoid granulomatosis (angioimmunoproliferative lesion) is an angiocentric and necrotizing, polymorphous lymphoid lesion that presents as multiple masses in the lung, involves skin and central nervous system (among other organs), may progress to histologically overt lymphoma, and is immunophenotypically predominantly a T cell process. Microscopically, a lymphohistiocytic infiltrate, including variable numbers of atypical cells, is present. Recent in situ hybridization and immunohistochemical studies have served to show EBV localized to the large cells. In addition, these atypical cells often show B cell immunophenotypic features and are present in a background of small, reactive T cells. It is therefore possible that lymphomatoid granulomatosis is a family of T cell rich lymphoproliferations driven by EBV infection of B cells and/or T cells, analagous to the EBV-associated, posttransplantation B cell lymphoproliferative disorders.
 ...
PMID:Pulmonary lymphoid disorders. 763 48

Lymphomatoid granulomatosis (LG) exhibits many similarities both clinically and pathologically to angiocentric T/NK cell lymphoma and was until recently considered to be part of the same disease spectrum. However, recent data indicate that LG is an EBV positive B cell proliferation associated with an exuberant T cell reaction. LG presents in extranodal sites, most commonly the lung (Katzenstein and Peiper, 1990). Other frequent sites of involvement include kidney, skin, central nervous system and liver. The pattern of necrosis in both LG and T/NK cell lymphoma is very similar, emphasizing the probable importance of EBV in mediating the vascular damage. Recent studies implicate the chemokines IP-10 and Mig in the pathogenesis of the vascular damage. Although the predominant infiltrating cells are T cells, the T cell receptor genes are not clonally rearranged. However, by VDJ polymerase chain reaction, approximately 60% of cases contain clonal rearrangements. EBV sequences can be localized to B cells and are clonal in most cases. Most patients with LG when carefully evaluated clinically have defects in cytotoxic T cell function and reduced levels of CD8+ T cells. LG is also common in many immunodeficiency states, such as AIDS, Wiskott-Aldrich syndrome and post-transplantation immunodeficiency. Thus, in many respects, LG resembles an EBV driven lymphoproliferative disorder. Some cases of LG regress spontaneously, but most patients require therapy. Treatment approaches have included cyclophosphamide and prednisone, aggressive combination chemotherapy and interferon alpha 2b, because of its antiviral, antiproliferative and immunomodulatory effects.
 ...
PMID:Lymphomatoid granulomatosis: pathogenesis, pathology and clinical implications. 954 95

Lymphomatoid granulomatosis (LYG) is a rare angio-destructive lymphoproliferative disorder (LPD) of uncertain etiology, with prominent pulmonary involvement. Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-associated B cell LPD with large numbers of background reactive T lymphocytes (T cell-rich B cell lymphoma). Although the disease frequently, but not exclusively, occurs in various immunodeficiency states, it has not been reported in association with the transient immunosuppression following autologous bone marrow/peripheral stem cell transplantation (ABM/PSCT). We describe a patient who developed lymphomatoid granulomatosis of the lung approximately 2 weeks after high-dose chemotherapy and autologous peripheral stem cell transplantation for multiple myeloma. Although molecular studies showed no evidence of EBV genome in the biopsy material, the serologic profile with high IgM titers was suggestive of primary EBV infection. Complete radiologic remission occurred following reconstitution of the patient's immune response after a 2-week course of ganciclovir treatment. Despite the apparently low frequency of LPD (both LYG and EBV-associated post-transplant lymphoma) in the ABMT setting, we believe that it should be considered in the differential diagnosis of patients whose clinical course following ABMT is complicated by fevers, in the absence of an identifiable infectious process.
 ...
PMID:Lymphomatoid granulomatosis following autologous stem cell transplantation. 1003 54

Three distinct entities are now covered by the definition of primary pulmonary clonal lymphoid proliferation. The aim of this review is to describe the pathophysiological, diagnostic, prognostic and therapeutic aspects of these three entities. Low-grade pulmonary B-cell lymphoma is the most frequent form of primary pulmonary clonal lymphoid proliferation. It arises from mucosa-associated lymphoid tissue. It is usually indolent and appears in the form of a chronic alveolar opacity. The prognosis is excellent, but treatment is controversial (simple monitoring, surgery or single-agent chemotherapy). High-grade pulmonary B-cell lymphoma is far rarer and usually occurs in individuals with an underlying disorder (e.g. immunodeficiency). The prognosis is poor and therapeutic options depend on the underlying disorder. The inclusion of lymphomatoid granulomatosis in the definition of primary pulmonary lymphomas is controversial. The clonal nature of the proliferation is very rarely demonstrated and extrapulmonary involvement is frequent (upper airways, skin, kidneys, central nervous system, etc.). The prognosis is extremely variable, with some authors reporting complete remission with steroids and cyclophosphamide and others reporting failure of combination chemotherapy.
 ...
PMID:Primary pulmonary lymphoma. 1235 56

Angiocentric lymphomatoid granulomatosis is a rare lymphoproliferative disease, mainly associated with pulmonary manifestation. Its origin is unknown, but Epstein-Barr virus may be one of the etiological factors. A 51-year-old male had an abdominal laparotomy in 1994 and a large granulomatous mass was removed from behind the cecum. No specific therapy was administered. In February 1998 multiple pulmonary lesions were found by X-ray and thoracoscopic biopsy was made. The histopathological diagnosis was angiocentric lymphomatoid granulomatosis. The patient received 6 cycles of CHOP chemotherapy, with which a complete remission was achieved. A consistent severe hypogammaglobulinaemia was detected, so the diagnosis of common variable immunodeficiency (CVID) was established. The diagnosed CVID was the probable causative factor of the angiocentric lymphomatoid granulomatosis. After the CHOP treatment, the patient is on intravenous immunoglobulin substitution and is well up to today.
 ...
PMID:Angiocentric lymphomatoid granulomatosis and severe hypogammaglobulinaemia. 1280 30

Lymphocytic interstitial pneumonia (LIP) is a clinicopathologic term that relates histologically to a dense interstitial infiltrate of mainly T cells, plasma cells, and histiocytes, with germinal centers often identified. Its precise etiology is unknown, but there are strong clinical associations with several autoimmune disorders, as well as both congenital and acquired immunodeficiency syndromes. It may overlap histologically with both extrinsic allergic alveolitis and nonspecific interstitial pneumonia, and therefore close clinical/radiological association is essential for diagnosis. LIP also overlaps clinically and histologically with follicular bronchitis/bronchiolitis, the latter showing reactive lymphoid hyperplasia with a peribronchiolar distribution predominantly comprising lymphoid follicles. LIP may also be histologically indistinguishable from nodular lymphoid hyperplasia and lymphomas arising from mucosa-associated lymphoid tissue (MALT) but can usually be differentiated via analysis of clinical and imaging data plus assessment of immunohistochemistry and gene rearrangement studies. Other entities include lymphomatoid granulomatosis, intravascular lymphomatosis, Castleman's disease, primary pleural lymphomas, primary effusion lymphomas, plasmacytomas, and secondary involvement by lymphoma, but these should all be readily distinguishable from lymphocytic interstitial pneumonia if all clinical, imaging, and histological data are apparent.
 ...
PMID:Lymphocytic interstitial pneumonia and other lymphoproliferative disorders in the lung. 1608 89


1 2 3 Next >>