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)

Persons infected with human immunodeficiency virus have an increased risk for development of high-grade, non-Hodgkin's lymphomas. Anaplastic large-cell Ki-1 lymphoma is a recently described lymphoid neoplasm characterized by cellular pleomorphism, a sinusoidal growth pattern, and Ki-1 epitope reactivity. This type of lymphoma is often mistaken for metastatic carcinoma, melanoma, or malignant histiocytosis. Although persons with acquired immunodeficiency syndrome frequently have non-Hodgkin's lymphoma at extranodal sites, the oral cavity and mandible, in particular, are unusual locations. We report two cases of anaplastic large-cell Ki-1 lymphoma that occurred in persons with the human immunodeficiency virus and with initial presentation as soft tissue masses of the posterior mandible. Immunocytochemical studies were positive for Ki-1 (CD30) in both cases. In situ hybridization for Epstein-Barr virus-deoxyribonucleic acid was positive with tumor cells in both cases. Flow cytometry on paraffin, formalin-fixed tissue revealed tetraploidy and high proliferative fractions that are characteristic of high-grade lymphomas. Intraoral presentation of rapidly enlarging, soft tissue masses may represent a high-grade non-Hodgkin's lymphoma in persons with the human immunodeficiency virus. Although rare, anaplastic large-cell Ki-1 lymphoma should be considered and requires immunocytochemical study to eliminate the possibility of other malignant conditions associated with the acquired immunodeficiency syndrome.
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PMID:Intraoral presentation of anaplastic large-cell Ki-1 lymphoma in association with HIV infection. 839 61

Most Epstein-Barr virus (EBV)-related infections in infants and children are asymptomatic or self-limited mild viral illnesses, but rare cases of a rapidly fatal disorder have been described. Failure of the cellular response to control EBV-related lymphoid proliferation leads to severe disease with multiple complications, including a fatal outcome or development of an EBV-driven, clonal lymphoid neoplasm. In this report we characterize 3 cases of fatal, nontransplant, or immunodeficiency-related EBV infection in very young children with immunophenotypic and molecular evidence of B/natural killer (NK)-T cell clonal expansion. An immunohistochemical staining panel included testing for B-cell antigen (CD20), and T/NK cell antigens including CD2, CD3, CD4, CD8, CD56, CD57, and TIA-1. T-cell and B-cell PCR clonality testing was performed on paraffin tissue specimens to identify clonal populations. The ages of these 3 patients ranged from 22 months to 4 years. Initial clinical presentations included pneumonia, abnormal liver function tests and fever, and lymphadenopathy. The 3 patients died within 17 to 72 days of presentation, and autopsy was performed on 1 patient. All cases demonstrated prominent atypical lymphoid or lymphohistiocytic infiltrates, and necrosis was present in 2 of the 3 cases. The atypical lymphocytes were positive for CD3 (cytoplasmic), CD2, CD8, TIA-1, and CD57 and negative for CD4. We molecularly identified B-cell clones in the 2 tested patients, who also showed evidence of hemophagocytosis. Fatal EBV infection is characterized by a morphologic spectrum with atypical lymphoid infiltrates and variable necrosis. Our molecular studies of these patients suggest a clonally-derived expansive process, most likely driven by EBV infection. Our results also suggest that development of clonality is associated with an aggressive clinical course and may be useful in predicting greater risk for fatal outcome. A high index of suspicion, coupled with appropriate serologic and molecular testing, aids in early recognition and diagnosis of these lymphoproliferative processes.
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PMID:Fatal non-transplant-related epstein-barr virus-associated atypical lymphoid proliferations in infants and children: a clinicopathologic study. 1847 5

Neoplastic lymphoid proliferation may arise from immune deficiency or disordered regulation of the immune system. Often the neoplasms are associated with viral agents, such as Epstein-Barr virus, human immunodeficiency virus, or human herpes virus 8. Lymphoproliferative diseases have been documented in a variety of primary immune disorders. The most commonly encountered neoplastic lesion is diffuse large B-cell lymphoma (DLBCL), although Hodgkin lymphoma (HL), Burkitt lymphoma, and peripheral T-cell lymphomas and/or leukemias have also been documented in rare instances. We report a case of a 6-year-old girl with unclassifiable primary immunodeficiency diagnosed with 2 different clones of DLBCLs and subsequently developed lymphocyte-depleted, classical HL. Both neoplasms were associated with Epstein-Barr virus. To the best of our knowledge, this is the first reported occurrence of primary immune disorder-associated lymphoproliferative disease with sequential development of DLBCLs and HL in a pediatric patient. Thorough surveillance is paramount for accurate assessment of the associated lymphoproliferative disease and in ascertaining likely transformation to, or de novo evolution of a different lymphoid neoplasm. This is also important in evaluating treatment response with appropriate therapeutic adjustments if clinically indicated.
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PMID:Subsequent development of diffuse large B-cell lymphomas and Hodgkin lymphoma associated with primary immune disorder in a 6-year-old female: a case report and review of the literature. 2157 47

Epidemiological studies suggest that retroviruses, including human immunodeficiency virus type 1, are associated with cardiomyopathy and myocarditis, but a causal relationship remains to be established. We encountered unusual cardiomyocyte hypertrophy and mitosis in Japanese native fowls infected with subgroup A of the avian leukosis viruses (ALVs-A), which belong to the genus Alpharetrovirus of the family Retroviridae and mainly induce lymphoid neoplasm in chickens. The affected hearts were evaluated by histopathology and immunohistochemistry, viral isolation, viral genome sequencing and experimental infection. There was non-suppurative myocarditis in eighteen fowls and seven of them had abnormal cardiomyocytes, which were distributed predominantly in the left ventricular wall and showed hypertrophic cytoplasm and atypical large nuclei. Nuclear chains and mitosis were frequently noted in these cardiomyocytes and immunohistochemistry for proliferating cell nuclear antigen supported the enhancement of mitotic activity. ALVs were isolated from all affected cases and phylogenic analysis of envSU genes showed that the isolates were mainly classified into two different clusters, suggesting viral genome diversity. In ovo experimental infection with two of the isolates was demonstrated to cause myocarditis and cardiomyocyte hypertrophy similar to those in the naturally occurring lesions and cardiac hamartoma (rhabdomyoma) in a shorter period of time (at 70 days of age) than expected. These results indicate that ALVs cause myocarditis as well as cardiomyocyte abnormality in chickens, implying a pathogenetic mechanism different from insertional mutagenesis and the existence of retrovirus-induced heart disorder.
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PMID:Cardiac pathology and molecular epidemiology by avian leukosis viruses in Japan. 2446 46

Intussusception of small bowel is considered a rare cause of bowel obstruction in adults accounting for only about 1% of bowel obstruction in adults. Intussusception in adults is uncommon with 95% cases of intussusceptions occurring in children. Adult intussusception from small intestinal lymphoma is also rare with only 36 cases reported in the literature between 2000 and 2011. Plasmablastic lymphoma (PBL) is an aggressive lymphoid neoplasm usually seen in the oral cavity in the clinical setting of human immunodeficiency virus (HIV) infection. Plasmablastic lymphoma of the small intestine is extremely rare. Here, we report a case of plasmablastic lymphoma of small bowel with ileoileal intussusception in an HIV-negative immunocompetent male patient.
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PMID:Ileo-ileal Intussusception and Bowel Obstruction Caused by Plasmablastic Lymphoma of Small Bowel- A Rare Entity in Rare Location. 2713 31