Two sibling cases of hydrops fetalis due to alloimmune anti-CD36 (Nak(a)) antibody
Department of Pediatrics, Hokkaido University, Sapporo, Hokkaidō, JapanThrombosis and Haemostasis (Impact Factor: 4.98). 02/2006; 95(2):267-71. DOI: 10.1267/THRO06020267
Two female sibling cases, who were born to a CD36 deficient mother, were presented with Coombs' test-negative hydrops. The alloimmune anti-CD36 (Nak(a)) antibody was accidentally found in the mother's serum after an episode of anaphylactic shock with thrombocytopenia, which occurred in an individual receiving fresh frozen plasma prepared from the mother's 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 1438-1449 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 mother's 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.
- Hematology, Immunology and Infectious Disease: Neonatology, Edited by Ohls and Yoder, 01/2008: chapter 9;
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ABSTRACT: Fetal and neonatal alloimmune thrombocytopenia (AIT) is a result of a parental incompatibility of platelet-specific antigens and the transplacental passage of maternal alloantibodies against the platelet antigen shared by the father and the fetus. It occurs in approximately 1 in 1000 live births and is the most common cause of severe thrombocytopenia in fetuses and term neonates. As screening programs are not routinely performed, most affected fetuses are identified after birth when neonatal thrombocytopenia is recognized. In severe cases, the affected fetus is identified as a result of suffering from an in utero intracranial hemorrhage. Once diagnosed, AIT must be treated antenatally as the disease can be more severe in subsequent pregnancies. While there have been many advances regarding the diagnosis and treatment of AIT, it is still difficult to predict the severity of disease and which therapy will be effective.Blood Reviews 02/2008; 22(1):33-52. DOI:10.1016/j.blre.2007.09.002 · 5.57 Impact Factor
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ABSTRACT: Anti-CD36 antibodies are known to cause a platelet refractory state. We describe a previously unreported case of a 16-year-old female sickle cell disease patient with anti-CD36 antibodies, detected on routine screen prior to hematopoietic stem cell transplantation (HSCT). CD36 platelet antigen typing was negative for both the patient and her HLA-identical donor sibling. Patient plasma was compatible with 48 of 49 apheresis platelets, which were untested and presumably positive for the CD36 antigen. The patient responded adequately to transfusion of crossmatch compatible platelets and successfully underwent HSCT. The presence of anti-CD36 antibodies does not exclude potential candidates from HSCT.Pediatric Blood & Cancer 03/2008; 50(3):660-2. DOI:10.1002/pbc.21180 · 2.39 Impact Factor
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