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UNUSUAL COMBINED IMMUNODEFICIENCY SYNDROME EXHIBITING KAPPA‐IGD PARAPROTEINEMIA, RESIDUAL GUT‐IMMUNITY AND GRAFT‐VERSUS‐HOST REACTION AFTER PLASMA INFUSION 1
Author(s) -
RUBINSTEIN A.,
RÁADL J.,
COTTIER H.,
ROSSI E.,
GUGLER E.
Publication year - 1973
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1973.tb08121.x
Subject(s) - paraproteinemia , medicine , immunoglobulin d , immunology , immunodeficiency , immunity , kappa , paraproteinemias , gastroenterology , pathology , multiple myeloma , antibody , immune system , b cell , linguistics , philosophy , monoclonal antibody , monoclonal
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.

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