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Satoru Okajima, Kazutoshi Cho, Hitoshi Chiba, Hiroshi Azuma, Toshiko Mochizuki, Miki Yamaguchi, Shin-ichiro Sato, Hisami Ikeda, Hideto Yamada, Hisanori Minakami, Tadashi Ariga, Kunihiko Kobayashi
Two sibling cases of hydrops fetalis due to alloimmune anti-CD36 (Naka) antibody
Keywords: Platelet immunology, gene mutations, immunity (auto-)
Two female sibling cases, who were born to a CD36 deficient mother, were presented with Coombs test-negative hydrops. The alloimmune anti-CD36 (Naka) antibody was accidentally found in the mothers serum after an episode of anaphylactic shock with thrombocytopenia, which occurred in an individual receiving fresh frozen plasma prepared from the mothers donated blood. The mother was then diagnosed as having type II CD36 deficiency, lacking CD36 on both platelets and monocytes, while both of her daughters were CD36 positive. Analyses of the CD36 gene revealed that the mother was a compound heterozygote for the CD36 gene mutation with a novel C T transition at nt 1366 in exon 12, corresponding to Arg386Trp, and a known 12bp deletion at nt 14381449 in exon 13. On the other hand, both patients, who showed half the normal level of CD36 on platelets and monocytes, were heterozygote with one mutation at Arg386Trp. The anti-CD36 antibody in the mother seemed to be responsible for the hydrops fetalis observed in her daughters, because the IgG isolated from the mothers serum showed suppressive effects on the CFU-E colony formation of CD34+ cells from a control donor. This is the first case report of hydrops fetalis caused by an alloimmune anti-CD36 antibody.
Thrombosis and Haemostasis, Schattauer
Print ISSN: 0340-6245
Volume: 95
Pages: 267 - 271