UNUSUAL COMBINED IMMUNODEFICIENCY SYNDROME EXHIBITING KAPPA‐IGD PARAPROTEINEMIA, RESIDUAL GUT‐IMMUNITY AND GRAFT‐VERSUS‐HOST REACTION AFTER PLASMA INFUSION1

Abstract

Summary A 12‐year‐old suffering from a lymphopenic severe CID with an unusual protracted clinical course is presented. His gut‐associated lympho‐epithelial system was apparently normal, in contrast to the IgA deficiency in other external fluids. In addition, an elevated kappa‐IgD was detected in his serum. Fresh plasma infusions from unrelated donors induced an accidental engraftment with a moderate non‐fatal GvHR.