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

We studied follicles in sections of lymph nodes and spleen from cynomolgus monkeys (Macaca fascicularis) after infection with simian immunodeficiency virus (SIVsm), by (immuno)histology and (immunogold) electron microscopy. Also isolated follicular dendritic cells (FDC) were investigated. Histology showed ranged from follicular hyperplasia to follicle fragmentation. FDC showed desmin and vimentin, characteristic of mesenchymal cells. Except for two animals who got experimental chemotherapy in the first postinfection period, the cells expressed SIV gag p28 protein. Electron microscopy showed SIVsm-like particles in the germinal centers. A number of cell types in the germinal center, including FDC, showed tubuloreticular structures, indicative of alpha-interferon synthesis during an antiviral response. In immunogold electron microscopy, SIV p28 label was observed on the surface of FDC, on SIVsm-like particles, and in the cytoplasm of macrophages. A relatively high density of CD8-positive cells (T cytotoxic-suppressor phenotype) was observed around and in germinal centers, especially areas depleted of FDC. Cells immunoreactive for serine esterase granzyme-B, a protein occurring in granules of cytotoxic cells, occurred around germinal centers, but not in germinal centers at areas where FDC and SIV p28 label localized. This argues against a role of cytotoxic T cells in mediating follicle destruction.
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PMID:Simian immunodeficiency virus (SIVsm) infection of cynomolgus monkeys: effects on follicular dendritic cells in lymphoid tissue. 149 52

The Authors report on a case of acute idiopathic giant cell myocarditis, which occurred in a young man without previous history of immunodeficiency or tumours, and displayed a rapidly fatal clinical course. Autoptic examination showed diffuse damage to the myocardium, with myocytolysis, granuloma formation and abundant giant cell reaction. No significant changes were observed in the other organs and systems . Immunohistochemistry revealed that the giant cells strongly reacted with the antibody KP1--raised to the macrophage-associated antigen CD68--whereas they did not stain with the monoclonal against the muscle-specific marker desmin. In the light of their findings and previous reports in the literature, the Authors discuss the possible origin of giant cells, along with the pathogenesis of the condition.
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PMID:Acute idiopathic interstitial giant cell myocarditis. A histological and immunohistological study of a case. 174 84

The intermediate filament (IF) subunit protein vimentin is efficiently cleaved in vitro by purified human immunodeficiency virus type 1 (HIV-1) protease. Immunological data confirm that identical sites are cleaved when vimentin is polymerized into filaments or occurs as protofilaments. Preformed filaments require 10 times more protease to achieve the same extent of cleavage seen with protofilaments, suggesting that the cleavage sites are partially masked in IFs. The primary cleavage gives rise to molecule lacking most of the tail domain and which not only remains in preformed filaments, but also is capable of polymerizing into essentially normal 10 nm filaments. However, these filaments of the vimentin primary cleavage product show a propensity to form large lateral aggregates. The three secondary cleavage products of vimentin additionally lack portions of the head domain, are almost quantitatively released from preformed filaments and are not capable of forming filaments de novo. These results confirm and extend previous data obtained with desmin and provide a limit for that portion of the tail domain of type III IF subunit proteins that play a role in IF formation and stability. Microinjection of HIV-1 protease into cultured human skin fibroblasts resulted in a large increase in the percentage of cells with an altered and abnormal distribution of vimentin IFs. Most commonly, the IFs were observed to have collapsed into a clump with a juxtanuclear localization. The efficient cleavage of vimentin observed in vitro and the ability of microinjected HIV-1 protease to alter IF distribution in vivo suggest that IF proteins may serve as substrates within HIV-1 infected cells and may play a role in viral infection.
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PMID:Effect of human immunodeficiency virus type 1 protease on the intermediate filament subunit protein vimentin: cleavage, in vitro assembly and altered distribution of filaments in vivo following microinjection of protease. 181 Dec 47

We report the occurrence of multiple leiomyomata and leiomyosarcomata in three children with the acquired immunodeficiency syndrome. The children were infected with human immunodeficiency virus as infants (one perinatally and two through transfusions) and each was profoundly immunosuppressed (absolute CD4 cell count, less than 75/mm3). They experienced multiple infections prior to the diagnosis of neoplasms at ages 4, 5, and 8 years, respectively. The tumors in each case were mainly involving the lungs and gastrointestinal tract. The smooth-muscle origin of the tumors was proved by light and electron microscopy and immunohistochemical staining for muscle-specific actin and desmin. Leiomyomata and leiomyosarcomata are rare tumors in childhood, suggesting a nonrandom association of tumors of smooth-muscle origin and the acquired immunodeficiency syndrome. We postulate that human immunodeficiency virus infection may play a direct or indirect role in tumor formation.
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PMID:Tumors of smooth-muscle origin in HIV-infected children. 234 29

A case of spindle-cell pseudotumor of the spleen due to nontuberculous mycobacteria in a patient with acquired immunodeficiency syndrome (AIDS) is described. The patient was a 55-year-old, human immunodeficiency virus-positive Haitian man who died of acute neurologic complications while on treatment for central nervous system toxoplasmosis. At autopsy, an enlarged multinodular spleen was noted. Histologic examination revealed coarse nodules of splenic parenchyma replaced by a dense spindle cell proliferation, admixed with scattered inflammatory cells. Immunostains showed strong cytoplasmic positivity of the spindle cells with MAC 387, HAM 56, and alpha-1-antichymotrypsin antibodies and negative staining for actin, vimentin, and S-100 protein antibodies. Ziehl-Neelsen stains revealed numerous elongated acid-fast bacilli within the cytoplasm of the cells that were occasionally lying free within the interstitium. The organisms also had a strongly positive reaction with antibodies to desmin intermediate filaments. Mycobacterial spindle-cell pseudotumor should be included in the differential diagnosis of conditions affecting the spleen in patients with AIDS.
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PMID:Mycobacterial spindle-cell pseudotumor of the spleen. 816 Jun 49

We report an 18-month-old Japanese girl with purely epithelioid leiomyosarcoma presenting as a huge intraabdominal mass. The patient had been well from birth and had shown no signs of immunodeficiency. She was negative for human immunodeficiency virus. Blood examination revealed elevated serum neuron specific enolase (NSE). Histologically, the tumor was comprised of solid growths of round or polygonal cells with vesicular nuclei and often vacuolated cytoplasm rich in glycogen. The tumor cells were positive for vimentin, NSE, and MIC2, and were negative for desmin and neurofilament. The age, clinical presentation, and histologic findings mostly favored Ewing's sarcoma/primitive neuroectodermal tumor. Silver stain, however, demonstrated well-developed reticulin fibers often outlining individual tumor cells. An expanded panel of immunostains showed that the tumor cells were intensely positive for smooth muscle actin, and ultrastructural study revealed abundant fine cytoplasmic filaments with focal subsarcolemmal densities, various amounts of glycogen, and irregularly arranged, thick basal lamina. The diagnosis of epithelioid leiomyosarcoma was made. Following reduction in tumor size by chemotherapy, the serum NSE level was normalized. From the surgical finding, the primary site was presumed to be the urachus or the urinary bladder dome. Although extremely rare, epithelioid leiomyosarcoma should be added in the list of differential diagnoses of pediatric "round cell tumors."
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PMID:Epithelioid leiomyosarcoma in a non-immunocompromised infant: additional differential diagnosis of pediatric "round cell tumors". 1104 12

A 31-year-old Japanese male was admitted to our hospital for investigation of an asymptomatic nodular lesion of the liver detected by abdominal ultrasonography (US) during a routine medical examination. Computed tomography (CT) revealed a single, hypovascular mass 35 mm in diameter, within the left lobe of the liver. The tumor demonstrated hypointensity on T1-weighted, and hyperintensity on T2-weighted magnetic resonance (MR) imaging. Hematological and biochemical investigations were normal. There were no abnormalities of the gastrointestinal or urinary tracts. A left lateral segmentectomy of the liver was performed. Pathological examination of the nodule revealed a primary leiomyoma of the liver, with positive immunohistochemical staining for vimentin and desmin antigens. Primary leiomyoma of the liver is rare, with the majority of cases associated with immunodeficiency disorders. This patient had no evidence of any underlying disease. Primary leiomyoma of the liver should be considered when a nodular lesion is found in a patient without evidence of viral hepatitis.
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PMID:A case of primary leiomyoma of the liver in a patient without evidence of immunosuppression. 1224 95

We describe the clinical, radiologic, surgical, and pathologic findings of a 29-year-old Peruvian human immunodeficiency virus-infected man with a primary parasellar meningeal leiomyosarcoma involving the left lesser esphenoidal wing and the cavernous sinus. Over a period of 13 months, he developed headache, vomiting, insomnia, and diplopia. Magnetic resonance imaging revealed a left parasellar extra-axial mass that was isointense in T1, hypointense in T2, and gadolinium-enhanced. The patient underwent subtotal resection of the tumor. The neoplasm was composed of spindle cells with smooth-muscle features. It showed moderate atypia, inconspicuous nucleoli, and scanty mitosis. No tumor necrosis was detected. The immunohistochemistry revealed strong positivity for vimentin, desmin, and smooth-muscle alpha-actin. A low-grade leiomyosarcoma was diagnosed. The in situ hybridization showed positive nuclear reactivity for Epstein-Barr virus-encoded RNA. The immunohistochemistry was negative for Epstein-Barr virus latent membrane protein 1. The main differential diagnosis of primary meningeal smooth-muscle tumors includes meningioma and peripheral nerve sheath tumors. Epstein-Barr virus has been demonstrated in most smooth-muscle tumors associated with acquired immune deficiency syndrome (AIDS). Primary meningeal smooth-muscle tumors, exceedingly rare neoplasms, remarkably affect young adults with AIDS. Comparatively, most AIDS-related visceral (nonmeningeal) smooth-muscle tumors have been reported in children. The permissiveness and tumorigenesis associated with Epstein-Barr virus may depend on the age of human immunodeficiency virus infection.
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PMID:Primary meningeal Epstein-Barr virus-related leiomyosarcoma in a man infected with human immunodeficiency virus: review of literature, emphasizing the differential diagnosis and pathogenesis. 1553 43

Multifocal submucosal stromal tumors were diagnosed in a 5.5-year-old rhesus macaque (Macaca mulatta) experimentally infected with simian immunodeficiency virus, strain SIVsmE660, and CD4+ T cell depleted. The animal was negative for simian retroviruses, SRV-1, -2, and -5. Polymerase chain reaction analysis of DNA from tumor and spleen tissue revealed abundant, preferential presence of retroperitoneal fibromatosis herpesvirus, the macaque homologue of the Kaposi sarcoma-associated herpesvirus (human herpesvirus-8), in the tumors. This was corroborated by demonstration of viral latent nuclear antigen-1 in the nuclei of a majority of the spindeloid tumor cells. Low levels of an additional macaque herpesvirus, rhesus rhadinovirus, were also detected in the spleen and tumor tissues. The spindeloid cells labeled positively for vimentin and CD117 but were negative for CD31, CD68, desmin, and smooth muscle cell actin. Collectively, these findings suggest a relation to but not absolute identity with simian mesenchymoproliferative disorders (MPD) or typical gastrointestinal stromal tumors (GISTs).
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PMID:Intestinal stromal tumors in a simian immunodeficiency virus-infected, simian retrovirus-2 negative rhesus macaque (Macaca mulatta). 1587 92

Polypoid tongue lesions arising after bone marrow transplantation have been described. Their etiopathogenesis has been unclear, as has their relationship to similar lesions arising in other settings of chronic immunodeficiency. We identified 12 polypoid lesions (from 8 immunosuppressed patients aged 6 months to 13 years) among all tongue lesions biopsied over the course of 13 years at our institution. Clinical history, histologic and ultrastructural features, special stains (Gram, Grocott methenamine silver, acid-fast bacilli, CD34, actin, desmin, human herpesvirus-8), in situ hybridization for Epstein-Barr virus, and cytogenetic features were studied. Immunocompromise was from bone marrow transplantation for severe combined immunodeficiency (n = 1) and acute lymphoblastic leukemia (n = 3), hypogammaglobulinemia (n = 2), 22q11 deletion syndrome (n = 1), and asthma therapy (n = 1). Histologic examination revealed fibrous stromal cores with squamous epithelial covering and various degrees of ulceration and accompanying inflammation and granulation tissue. In 2 patients lesions were multiple in number. Fibroblasts were variably positive for smooth muscle actin and desmin and negative for CD34. Special stains, immunohistochemistry, in situ hybridization, and ultrastructural examination identified no organisms except occasional surface bacteria. The tongue lesion from 1 patient with Down's syndrome showed t(2;9)(p11;q34)+21 (translocation not seen in peripheral blood). Another patient had constitutional del 22q11. All transplant patients had Philadelphia chromosome-positive acute lymphoblastic leukemia (ALL) (translocations involving 9q34 and 22q11). Patients with congenital immunosuppression had polyps arise at significantly younger ages than did patients with acquired immunosuppression. Immunosuppression-related lingual polyps are a fibroproliferative process occurring in patients with bone marrow transplantation and other immune-deficient conditions. Our findings indicate that these polyps are driven by both immunosuppression and chromosomal rearrangement.
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PMID:Immunosuppression-related fibroproliferative polyps of the tongue. 1763 30


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