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Hemophagocytic Lymphohistiocytosis in the Premature Neonate: A Case Study

Woods, Amanda G. MSN, RN, NNP-BC1; Woods, Christopher W. MSN, RN, NNP-BC2

Section Editor(s): KING, CHERYL MS, CCRN

Advances in Neonatal Care:
doi: 10.1097/ANC.0b013e3181c1fff7
Case of the Month

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting from an abnormal proliferation of histiocytes within the body's tissues leading to an ineffective immune response. Typically, HLH is characterized by fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and hemophagocytosis. However, the premature infant with HLH may present differently making diagnosis of the disease cumbersome. If an infant is born with ascites, cytopenias, hypofibrinogenemia, and hepatosplenomegaly, a diagnosis of HLH cannot be ruled out. In addition, premature infants oftentimes will not present with fever because they are kept normothermic from ambient sources. Reports of premature infants with HLH in the literature are rare. This is a case presentation of a 27-week-gestation female with a family history of HLH.

Author Information

1Women's Hospital, Greensboro, North Carolina, and 2Wake Forest University Baptist Medical Center, Winston Salem, North Carolina.

Address correspondence to Amanda G. Woods, Women's Hospital of Greensboro, 801 Green Valley Rd, Greensboro, NC 27408;

© 2009 National Association of Neonatal Nurses