X-linked Lymphoproliferative Disease

Abstract
• Objective. —To study the inheritance of the X-linked lymphoproliferative disease (XLP) locus in a German family. Design. —Haplotype segregation analysis. Setting. —Departments of Pediatrics and Human Genetics, University of Würzburg and University of Ulm, Federal Republic of Germany. Participants. —Fourteen members of a family with XLP. Interventions. —None. Measurements/Main Results. —Using molecular genetic techniques, we diagnosed the XLP status of the members of a German family. Restriction fragment length polymorphism analysis with closely linked polymorphic X-chromosomal DNA markers (Xq25-q27) revealed XLP carrier status in a female infant. Moreover, the XLP mutation was suggested to have occurred in the germ cell of the grandfather. Conclusion. —This is the first report of a paternally inherited XLP mutation. The recurrence risk for germ cell mosaicism in XLP may be similar to that of X-linked Duchenne muscular dystrophy. (AJDC. 1993;147:1303-1305)