A case involving a newborn with thrombocytopenia illustrated the importance of considering all possible fetal and neonatal alloimmune thrombocytopenia (FNAIT) differential diagnoses, including hemophagocytic lymphohistiocytosis (HLH). The case was recently published in Transfusion Medicine.
“ We report an infant aged less-than-four months with an atypical hyperimmune response to a viral infection, who developed anti-E and anti-K of IgM class following allogeneic RBC exposure Anti-A and anti-B were also detectable relatively early,” the authors wrote.
The patient was born after 38 weeks of gestation and had an uncomplicated pregnancy. Apart from diabetes mellitus type 2 control with metformin, the mother had no other significant previous illnesses. During the first 4 days after birth, the patient presented yellowish coloration of the skin and eyes, but this resolved spontaneously, and he was discharged home.
At 44 days of age, his parents took him to the emergency room for a fever, laboratory testing upon admission revealed a low platelet count (thrombocytopenia) and anemia. The combination of anemia and thrombocytopenia in newborns could be suggestive of FNAIT, however, symptoms onset 44 days after birth and the presence of fever were indicative of an alternate diagnosis.
Physicians performed a viral panel, which turned out positive for cytomegalovirus (CMV), and diagnosed postnatal CMV infection complicated with bone marrow suppression. The patient underwent several plasma and platelet transfusions, and after confirming an improvement in hemoglobin and platelet levels, the patient was discharged home.
Two weeks later, he continued with fever and was readmitted with lower platelet and hemoglobin levels than at the initial admission. Therefore, he underwent a bone marrow aspirate which revealed findings consistent with HILL and subsequent genetic testing confirmed the diagnosis.
After undergoing treatment with corticosteroids his platelet and hemoglobin levels improved and the patient is currently doing well. However, due to the initial transfusions, the patient developed antibodies against red blood cell antigens E and K, which puts him at risk for adverse transfusion reactions if he needs one in the future.
“In the presence of significant inflammation (as in other circumstances too), transfusions should be used only when clinically justifiable,” the authors wrote. “ This may help to minimize alloimmunization risk, which has long-term consequences,”
About HLH
HLH is a potentially life-threatening condition in which immune cells, such as cytotoxic T lymphocytes, natural killer cells, and macrophages, are excessively activated during infection.
The disease can produce symptoms like anemia, thrombocytopenia, fever, enlarged liver (hepatosplenomegaly), enlarged spleen (splenomegaly), and elevated triglycerides. It is diagnosed based on the fulfillment of specific diagnostic criteria and treated with immunosuppressive therapy.
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