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Long‐term survival and preservation of natural killer cell activity in a xeroderma pigmentosum patient with spontaneous regression and multiple deposits of malignant melanoma
Author(s) -
ANSTEY A.V.,
ARLETT C.F.,
COLE J.,
NORRIS P.G.,
HAMBLIN A.S.,
LIMB G.A.,
LEHMANN A.R.,
WILKINSON J.D.,
TURNER M.
Publication year - 1991
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.1991.tb14754.x
Subject(s) - xeroderma pigmentosum , melanoma , medicine , term (time) , regression , natural (archaeology) , oncology , cancer research , dermatology , biology , genetics , dna repair , dna , psychology , paleontology , physics , quantum mechanics , psychoanalysis
Summary A 67‐year‐old man with xeroderma pigmentosum (XP) originally presented with malignant melanoma at the age of 28 years. This recurred 22 years later and subsequently numerous primary and secondary melanomas developed on the skin, several of which underwent spontaneous regression. Despite a marked lymphopenia, the proportion of natural killer cells was elevated and it is proposed that this led to the regression of the melanomas. Skin‐derived fibroblasts from the patient were more sensitive to UVC (D 10 ε3 J/m –2 ) than those from normal individuals (D 10 – 15 J/m –2 ).The fibroblast culture was shown to be defective in excision repair with <10% of residual activity compared with controls. No assignment to a complementation group has yet been made. There was an elevated frequency of mutants resistant to 6‐thioguanine in the circulating T lymphocytes.