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The effects of radiation therapy for hodgkin's disease in a child with ataxia telangiectasia. A clinical, biological and pathologic study
Author(s) -
Pritchard Jon,
Sandland M. R.,
Breatnach F. B.,
Pincott J. R.,
Cox R.,
Husband Peter
Publication year - 1982
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19820901)50:5<877::aid-cncr2820500513>3.0.co;2-u
Subject(s) - medicine , ataxia telangiectasia , disease , radiation therapy , telangiectasia , pediatrics , lymphoma , girl , hodgkin's lymphoma , ataxia , immune system , dermatology , pathology , immunology , dna , psychology , developmental psychology , genetics , dna damage , psychiatry , biology
Stage I lymphocyte‐predominant Hodgkin's disease was diagnosed in a 44‐month‐old girl. Although immune deficiency was suspected and IgA deficiency demonstrated, the diagnosis of an ataxia‐telangiectasia (AT)‐like syndrome was not confirmed until eight weeks later when results of studies on the radiosensitivity of cultured skin fibroblasts were available. The child had none of the usual physical stigmata of AT. Severe acute radiation damage followed the treatment of this child with standard doses of radiation therapy. Clinical, pathologic, and radiobiologic correlations are drawn. The diagnosis of a malignant lymphoma disorder in children under the age of five should alert clinicians to the possibility of immune deficiency and, even in the absence of classical physical signs, to AT in particular. Suggestions for the management of future similar cases are put forward.