An unusual family cancer syndrome manifested in young siblings

Article Abstract:

Some genetic disorders, such as retinoblastoma, involve the inheritance of a gene that results in a specific malignancy. Other genetic disorders, such as neurofibromatosis, include several forms of cancer within a spectrum of symptoms. However, there are rare families, known simply as 'cancer families', in which the only obvious feature is the occurrence of cancer in several closely-related family members. In cancer families, there is a high rate of sibling involvement, which usually occurs at a young age, and sometimes several cancers develop within a single affected individual. If the common and widely studied genetic causes of cancer are excluded, the most common malignancies among cancer families are brain tumors and sarcomas. The authors describe an unusual cancer family in which germ cell cancers seemed to predominate; this family appears to be the first in which germ cell cancers have been found to associate with embryonal sarcoma. The three patients, all girls, were born to a woman who had developed an ovarian mass as a teenager. The three daughters developed cancer at 10, 11, and 15 years of age. Two daughters developed abdominal masses. In one case, the mass was found to be an embryonal carcinoma; in the other, the mass proved to be a teratoma with foci of embryonal carcinoma cells present. The third daughter developed a mass in her neck that was not associated with other symptoms. Biopsy revealed the mass to be an embryonal rhabdomyosarcoma, a form of muscle cancer. All three patients were treated with surgery and chemotherapy; the neck mass was treated with radiation as well. All three patients were long-term survivors and the eldest became pregnant herself. Two brothers and two sisters from the same family had not developed any signs of malignant disease. The similar embryonic character of the tumors in all three children and a history of an ovarian mass in the mother strongly suggests that these cancers were the result of genetic influences. Although the children were all healthy the time of this report, it will be important to carefully monitor all of the siblings for other cancers. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Cancer, Cancer patients, Cancer genetics, Familial diseases, Tumors, Embryonal, Embryonal tumors

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Expression of the multidrug transporter, P-glycoprotein, in acute leukemia cells and correlation to clinical drug resistance

Article Abstract:

Many cancer patients who fail to respond to chemotherapy or who suffer relapses have cancer cells with drug resistance. Although leukemia is usually quite amenable to treatment with chemotherapeutic agents, the development of drug resistance is also common among leukemia patients. Leukemic cells present an advantage to researchers; living cancer cells are as easy to obtain as drawing a blood sample. Consequently, leukemic cells are commonly used in the examination of the biochemical basis for drug resistance. There has been some indication that at least a portion of drug resistance may be related to the presence of a glycoprotein on the cell surface which has been dubbed P-glycoprotein. Some investigators believe that this protein is part of a pump apparatus that ejects anticancer drugs from the cells. The gene for P-glycoprotein is called MDR1 for multiple drug resistance. There have, however, been conflicting reports about the prevalence of P-glycoprotein on the cells of leukemia patients with clinical drug resistance. To evaluate the role of P-glycoprotein in clinical drug resistance, blood cells from 18 patients with acute myelogenous leukemia and 13 patients with acute lymphoblastic leukemia were chemically analyzed. The results revealed that the patients who were found to have P-glycoprotein on their leukemic cells at the time of initial presentation were much less likely to achieve a response to chemotherapy than those who did not. Of six relapsed patients, four were found to have the P-glycoprotein; it is noteworthy that two of these patients did not have the P-glycoprotein on initial presentation. About 80 percent of the patients in this study had P-glycoprotein, which is somewhat higher than the prevalence reported by other researchers. Furthermore, it indicates that P-glycoprotein cannot be the only determinant of drug resistance, as some patients without P-glycoprotein were found to be resistant. Nevertheless, the findings suggest that P-glycoprotein should be considered an important marker for drug resistance. (Consumer Summary produced by Reliance Medical Information, Inc.)

Causes of, Biological transport, Active, Active biological transport, Leukemia, Chemotherapy, Drug resistance

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Hairy cell leukemia in two siblings: a human leukocyte antigen-linked disease?

Article Abstract:

Hairy cell leukemia, which is characterized by "hairy" cytoplasmic projections from the leukemic cells, is a rare disorder which sometimes occurs within families. While the familial occurrence suggests a genetic component to this disease, there are too few examples to have worked out the details of its inheritance pattern. One study has shown that three siblings with hairy cell leukemia shared the A1, B7 HLA haplotype, but there was not enough information to rule out a chance relationship. HLA haplotypes are the genes inherited from a parent for the human leukocyte antigens (HLA). These genes play a major role in the rejection of transplanted tissue, and play some role in a number of diseases which, in general, relate in some way to the immune response. Although the majority of genetic disorders are not related to HLA type, the ready availability of testing reagents has made HLA typing routine for many laboratory investigations, particularly of diseases involving white blood cells. When two brothers where found to have hairy cell leukemia, they were tested for HLA type, as were two unaffected siblings. The two affected siblings both had inherited the HLA haplotype consisting of the genes A2; Bw6, Bw62(15); Cw1; DR4, DRw53; and DQw3. A review of the literature showed that none of these genes are common to all five familial occurrences of hairy cell leukemia reported so far. Although a subtle relationship to the HLA type cannot be ruled out, there is no evidence clearly pointing to a genetic relationship between HLA type and hairy cell leukemia. (Consumer Summary produced by Reliance Medical Information, Inc.)

HLA histocompatibility antigens, HLA antigens, Leukemia, Hairy cell, Hairy cell leukemia, Histocompatibility antigens

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