Hydrops fetalis caused by homozygous alpha-thalassemia and Rh antigen alloimmunization: report of a survivor and literature review.
Hematologic causes of hydrops fetalis include homozygous alpha-thalassemia and immune hemolytic anemias. We report the case of a boy with hydrops fetalis who had homozygous alpha-thalassemia and alloimmune hemolytic anemia due to anti-E and anti-C blood group antibodies. He received intrauterine red blood cell transfusions and postnatal chronic transfusion with iron chelation therapy. A non-myeloablative sibling stem cell transplant failed. He is now 5 years and 6 months of age, hypothyroid with short stature, but in overall good health. He is one of the oldest reported homozygous alpha-thalassemia survivors and, to our knowledge, the only survivor with immune- and nonimmune-induced hydrops fetalis.
Joshi
Divya-Devi
DD
Pediatric Hematology/Oncology, Marshfield Clinic, Marshfield, Wisconsin 54449, USA. joshi.divya-devi@mashfieldclinic.org
Nickerson
H James
HJ
McManus
Michael J
MJ
D000328:Adult; D002675:Child, Preschool; D005260:Female; D006720:Homozygote; D006801:Humans; D015160:Hydrops Fetalis / Q000209:etiology* / Q000235:genetics / Q000276:immunology; D007231:Infant, Newborn; D008297:Male; D012203:Rh Isoimmunization / Q000150:complications* / Q000235:genetics / Q000276:immunology; D017741:Survivors; D017085:alpha-Thalassemia / Q000150:complications* / Q000235:genetics / Q000276:immunology