Summary

Two female sibling cases, who were born to a CD36 deficientmother, were presented with Coombs’ test-negative hydrops.The alloimmune anti-CD36 (Naka ) antibody was accidentallyfound in the mother’s serum after an episode of anaphylacticshock with thrombocytopenia, which occurred in an individualreceiving fresh frozen plasma prepared from the mother’s donatedblood.The mother was then diagnosed as having type IICD36 deficiency, lacking CD36 on both platelets and monocytes,while both of her daughters were CD36 positive. Analysesof the CD36 gene revealed that the mother was a compoundheterozygote for the CD36 gene mutation with a novel C → T transition at nt 1366 in exon 12, corresponding toArg386Trp, and a known 12bp deletion at nt 1438–1449 in exon13. On the other hand, both patients, who showed half the normallevel of CD36 on platelets and monocytes, were heterozygotewith one mutation at Arg386Trp.The anti-CD36 antibodyin the mother seemed to be responsible for the hydrops fetalisobserved in her daughters, because the IgG isolated from themother’s serum showed suppressive effects on the CFU-E colonyformation of CD34+ cells from a control donor.This is thefirst case report of hydrops fetalis caused by an alloimmune anti-CD36 antibody.