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April 2, 1982

Fatal Lymphohistiocytic Proliferation Owing to Epstein-Barr Virus

JAMA. 1982;247(13):1811. doi:10.1001/jama.1982.03320380017015

To the Editor.—  The report by Cividalli et al (1981;246:1917) proposed that graft-v-host reaction led to fatal lymphohistiocytic proliferation in a 6-year-old boy exhibiting XX/XY lymphoid chimerism. In addition to the chimerism, possible immune deficiency to Epstein-Barr Virus (EBV) ought to be considered in the etiology of this disorder. Our initial report of males with X-linked lymphoproliferative syndrome (XLP) assumed that death in three brothers who had had infectious mononucleosis was caused by familial lymphohistiocytosis. Marked activation of histiocytes in the tissues of the boys led us to this erroneous conclusion.1,2The chimeric boy described by Cividalli et al is reminiscent of the New World monkey, a marmoset that often is an XX/XY chimera highly susceptible to EBV-induced lymphoproliferative disease.3 Both marmosets and males with XLP show deficient antibody responses to EBV.4The diseases in immunodeficient patients provoked by this virus are comparable with that described in