TY - JOUR
T1 - Association of HLA-DR5 with mycosis fungoides
AU - Safai, B.
AU - Myskowski, P. L.
AU - Dupont, B.
AU - Pollack, M. S.
N1 - Funding Information:
Mycosis fungoides (MF) is an uncommon neoplasm of the lymphoreticular system with clinical, histologic, and immunologic features distinct from other forms of lymphoma. Substan- Manuscript received May 5, 1982; accepted for publication October 25, 1982. Supported in pat·t by NIH Grants CA-08748 and CA-22507 and by grants from Chesebrough-Ponds Inc, Dickson, Mirsky and Robert S. Sinn Fund, and the Special Projects Committee of the Society of Memorial Sloan-Kettering Cancer Center. Reprint requests to: Dr. Bijan Safai, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, New York 10021. Abbreviations: CTLC: cutaneous T-celllymphoma MF: mycosis fungoides SS: Sezary's syndrome tially greater understanding of MF and Sezru·y syndrome (SS) was made possible by the recent advances in the area of lymphocyte biology which have permitted accurate chru·acterization of the neoplastic cells of MF and SS as helper type T lymphocyte, thus allowing classification of these disorders as cutaneous T-celllymphoma (CTCL) [1]. The classical form of the disease manifests initially in the skin and pmsues a slowly progressive course with eventual involvement of the internal organs resulting in the death of the patient. While the cause of CTCL remains unclear, several investigators have suggested a role for various environmental factors in the pathogenesis of the disease. Industrial exposure has been linked to the disease at a statistically significant level by Cohen [2]. Further, Fischmann et al, found that 91% of MF patients had had multiple exposure to potentially cru·cinogenic chemicals [3]. Recently Poiesz et al, implicated viral infection as another possible contributory factor by their finding of type C retrovirus particles in the cultmed lymphocytes of MF patients [ 4]. Aside from the environmental factors and the possible role of retroviruses, a genetic susceptibility has also been suggested as playing a part in the pathogenesis of MF. While no characterizable mode of inheritance or ethnic group predisposition has been observed in the disease, an increased frequency of fiTst degree relatives with lymphoma and leukemia has been noted by some authors [5]. CTCL has been reported to occm in a cluster pattern in some families [6]. In addition, prior studies have suggested an increased occurrence ofHLA antigens (Awl9, BB, Cw) in MF patients [7]. These observations have led us to further investigate the role of genetic factors as determined by HLA typing in the disease. Ow-results indicate that the HLA genetic mru·ker DR5 is significantly increased in patients with MF and suggest that HLA-linked genetic susceptibility factors may play a role in the initiation of the disease.
PY - 1983
Y1 - 1983
N2 - Mycosis fungoides (MF) and Sezary syndrome (SS) are uncommon neoplasms of the lymphoreticular system with distinct clinical, histologic, and immunologic features. Based on the thymus-derived nature of the neoplastic cells, MF and SS are both classified as cutaneous T-cell lymphoma. While substantially greater understanding of MF and SS has been made possible, the extent mechanism for the initiation of either disease is still unknown. The possible involvement of environmental factors as well as viral etiology, i.e., retroviruses, has been suggested. In order to investigate the possible role of HLA-associated variations in genetic susceptibility, 74 patients with histologically documented MF were typed for HLA-A, -B, and -C antigens. Half of these patients were also typed for HLA-DR antigens. An increase in DR5 was the only statistically significant deviation in HLA antigen frequencies in these patients (53% in MF as compared with 20% in controls). An increased frequency of HLA-DR5 has also been associated with scleroderma and juvenile rheumatoid arthritis both of which have immunologic alterations. Also HLA-DR5 has been associated with renal cell carcinoma and Kaposi's sarcoma. The association of MF with DR5 suggests that some individuals with the DR5 antigen may be at higher risk for virally initiated and/or neoplastic diseases possibly through an HLA-linked defect in the immune system.
AB - Mycosis fungoides (MF) and Sezary syndrome (SS) are uncommon neoplasms of the lymphoreticular system with distinct clinical, histologic, and immunologic features. Based on the thymus-derived nature of the neoplastic cells, MF and SS are both classified as cutaneous T-cell lymphoma. While substantially greater understanding of MF and SS has been made possible, the extent mechanism for the initiation of either disease is still unknown. The possible involvement of environmental factors as well as viral etiology, i.e., retroviruses, has been suggested. In order to investigate the possible role of HLA-associated variations in genetic susceptibility, 74 patients with histologically documented MF were typed for HLA-A, -B, and -C antigens. Half of these patients were also typed for HLA-DR antigens. An increase in DR5 was the only statistically significant deviation in HLA antigen frequencies in these patients (53% in MF as compared with 20% in controls). An increased frequency of HLA-DR5 has also been associated with scleroderma and juvenile rheumatoid arthritis both of which have immunologic alterations. Also HLA-DR5 has been associated with renal cell carcinoma and Kaposi's sarcoma. The association of MF with DR5 suggests that some individuals with the DR5 antigen may be at higher risk for virally initiated and/or neoplastic diseases possibly through an HLA-linked defect in the immune system.
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U2 - 10.1111/1523-1747.ep12553615
DO - 10.1111/1523-1747.ep12553615
M3 - Article
C2 - 6601679
AN - SCOPUS:0020623846
VL - 80
SP - 395
EP - 397
JO - Journal of Investigative Dermatology
JF - Journal of Investigative Dermatology
SN - 0022-202X
IS - 5
ER -