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Hemophagocytic Lymphohistiocytosis in the Premature Neonate

Woods, Christopher W. MSN, RN, NNP-BC1,2; Bradshaw, Wanda T. MSN, RN, NNP-BC1,3; Woods, Amanda G. MSN, RN, NNP-BC4


doi: 10.1097/ANC.0b013e3181c20010
Beyond the Basics

Hemophagocytic lymphohistiocytosis (HLH), a rare disease, results in pathological findings secondary to an abnormal proliferation of activated lymphocytes and histiocytes (tissue macrophages) and is lethal unless identified and adequately treated. Clinical features of HLH include fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, elevated blood levels of ferritin, lymphadenopathy, skin rash, jaundice, and edema. Often, the symptoms of HLH are misinterpreted as infection, resulting in inadequate treatment and death. Several case studies of premature neonates with HLH have recently been published. Therapeutic guidelines for HLH exist and, when identified, HLH in the premature infant can be successfully treated resulting in resolution of symptoms.

1Duke University School of Nursing, Durham, North Carolina, 2Wake Forest University Baptist Medical Center, Winston Salem, North Carolina, 3Duke University Health System, Durham, North Carolina, and 4Women's Hospital, Greensboro, North Carolina.

Address correspondence to Christopher W. Woods, MSN, RN, NNP-BC, Wake Forest University Baptist Medical Center, Brenner Children's Hospital, Winston-Salem, NC 27157;

© 2009 National Association of Neonatal Nurses