A newborn with a rare and life-threatening combination of Jka hemolytic disease of the fetus and newborn (HDFN) and fetal and neonatal alloimmune thrombocytopenia (FNAIT) was successfully treated, highlighting the importance of early detection and targeted care, according to a case report published recently in BMC Pediatrics.
The infant’s recovery depended on timely blood transfusions and immunoglobulin therapy. The newborn boy was delivered at 39 weeks via emergency cesarean section after decreased fetal movement and an unresponsive non-stress test prompted urgent intervention.
At birth, he showed no breathing or muscle tone and had an extremely low Apgar score. Immediate resuscitation and ventilation were needed. Doctors found the newborn severely anemic, with a hemoglobin level of just 30.8 g/L, a critically low platelet count of 13 × 10⁹/L and signs of metabolic acidosis and shock. His condition declined rapidly, requiring emergency blood transfusions.
“In general, HDFN secondary to antibodies against non-ABO/Rh antigens should be considered in cases of hemolytic anemia in a newborn in the absence of ABO or Rh incompatibility,” the authors wrote. “Prompt maternal and neonatal DAT testing and antibody identification are essential for diagnosis, followed by aggressive treatment to minimize the risk of adverse outcomes.”
Read more about FNAIT signs and symptoms
Further testing confirmed that both mother and baby carried anti-Jka antibodies, indicating Jka hemolytic disease. At the same time, both tested positive for platelet antibodies, confirming HDFN. The newborn’s anemia and thrombocytopenia improved with three red cell transfusions and intravenous immunoglobulin, which raised his hemoglobin and platelet levels and stabilized his condition.
Upper gastrointestinal bleeding and dangerously low platelet levels on day 2 responded to gastric lavage, topical thrombin, and continued intravenous immunoglobulin treatment. By day 4, his levels of bilirubin peaked at 221 umol/L and were managed with phototherapy. Gradually, his color, oxygen levels, and lab values normalized. No abnormalities were seen in cranial MRI or hearing tests. He was discharged on day 8, and follow-up showed normal development.
The mother had shown no signs of thrombocytopenia or anemia in her previous pregnancy and had not been screened for irregular antibodies during this one. Her platelet count was normal before delivery, but the irregular antibodies test was positive on the day of delivery. This case suggests that routine antibody screening in later pregnancies might help identify risks earlier.
Only 14 previous cases of neonatal Jka hemolytic disease have been reported in the medical literature, and none involved HDFN. This case emphasizes the need for greater awareness among clinicians, as missed or delayed diagnosis of rare but severe conditions such as this can be fatal without immediate treatment. For families, this underscores the importance of prenatal monitoring and follow-up if there’s a history of unusual symptoms in pregnancy or a complicated delivery.
