Immunologic observations in close relatives of two sisters with mammary Burkitt's lymphoma: mammary Burkitt's lymphoma in sisters

Article Abstract:

Burkitt's lymphoma is endemic in Africa, where it appears to be the result of infection with Epstein-Barr virus (EBV). The lymphoma appears to develop after EBV infection in children whose cellular immune system has been suppressed by malaria. In contrast, the isolated cases of Burkitt's lymphoma elsewhere in the world appear to be largely unrelated to EBV infection. However, little is known about whether these lymphoma patients also have some abnormalities of cellular immunity. In Africa, Burkitt's lymphoma commonly affects the jaw; in Western nations, abdominal organs are more likely to be affected; and the breast is rarely involved. Two unusual cases are presented of two sisters who both developed Burkitt's lymphoma of the breast. Immunological testing revealed that the patients' mother and one of the patients' two healthy sisters had increased amounts of IgG antibody against the viral capsid antigen (VCA) of Epstein-Barr virus. The mother also had increased amounts of IgA antibody against VCA, and elevated antibody titers against EBV nuclear antigen and EBV early antigen as well. The antibody titers against EBV were not increased in the patients themselves. Furthermore, genetic analysis could not demonstrate EBV DNA in the lymphoma cells. The authors point out a parallel between these findings and those in families with X-linked lymphoproliferative disease. In these families, boys are likely to inherit the disorder from their carrier mother. The boys may develop a B-cell lymphoma as a consequence of EBV infection, but do not have elevated antibodies when tested. The carrier mother of such boys, as well as any sisters that might carry the gene, have high antibodies against this virus. The presence of elevated antibodies against EBV is thought in some cases to indicate a defect in the immune defenses mediated by T cells. The similarity of the immunological findings in these two reported cases of Burkitt's lymphoma of the breast suggest that similar mechanisms might be at work in this instance. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Physiological aspects, Genetic aspects, Burkitt's lymphoma, Burkitt lymphoma

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Lewis blood group antigens (a and b) in human breast tissues: loss of Lewis-b in breast cancer cells and correlation with tumor grade

Article Abstract:

As their name implies, blood group antigens were first discovered on blood cells. However, blood group antigens are expressed by other tissues as well. The Lewis blood group antigens, like the more famous antigens of the ABO system, are not proteins but complex sugar modifications added onto cell surface proteins. A study was undertaken to determine if the expression of two Lewis system antigens, Le-a and Le-b, is altered in breast cancer. Six specimens of either normal breast tissue or benign proliferative disease were microscopically examined, as were one case of Paget's disease, two cases of mucinous carcinoma, one case of papillary carcinoma, and 25 cases of invasive ductal carcinoma. Using specific monoclonal antibodies to identify expression of Le-a and Le-b, it was found that the normal and benign breast tissue contained Le-b. Le-a was found in only one specimen, and even in that specimen it was not uniformly distributed, but was only in one area. Only five of the invasive ductal carcinomas contained Le-b, and only one of the less common breast cancers contained this antigen. None of the cancerous specimens contained Le-a. These results indicate that the expression of the Lewis a antigen is uncommon in breast tissue, whether healthy or diseased. In contrast, expression of the Le-b antigen appears to be a characteristic of healthy breast tissue. While cancerous breast tissue can express the Lewis b antigen as well, the malignant transformation of breast cancer cells involves the loss of the Lewis b antigen in the majority of cases. (Consumer Summary produced by Reliance Medical Information, Inc.)

Diagnosis, Blood groups, ABO blood-group system, Pathology, Immunologic diseases

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Primary nasal-paranasal oropharyngeal lymphoma in the pediatric age group

Article Abstract:

Among a total of 208 pediatric patients with non-Hodgkin's lymphoma seen over a 15-year period, 20 patients developed their primary tumor in the nasal or oral cavities. Although most reports of nasal, paranasal, oral, and pharyngeal (NPOP) lymphoma include pediatric cases along with adult cases, the clinical presentation, histological appearance, survival, and recurrence patterns suggest that pediatric NPOP lymphoma may be considered a distinct clinical entity. Unlike adults, most of the pediatric patients had B cell lymphomas of the diffuse undifferentiated or small cell uncleaved types. The event-free survival among the 20 patients was 75 percent, with a median observation of over 99 months. Although many systems for staging tumors concentrate on the spread of tumor outside of the primary site, staging by these systems did not correlate well with outcome. However, when size of the original tumor was included in the staging criteria, the correlation was better. Among the patients studied, recurrences were early, suggesting that among pediatric patients, dissemination of the tumor has occurred even in apparently early stages, and that, in addition to radiotherapy, intensifying chemotherapy may produce better results. (Consumer Summary produced by Reliance Medical Information, Inc.)

Prognosis, Demographic aspects, Non-Hodgkin's lymphomas

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