Cytomegalovirus-Induced Hemophagocytic Lymphohistiocytosis in an Extreme Preterm Infant: Recognition and Therapy Leading to Recovery

Adv Neonatal Care. 2017 Apr;17(2):91-95. doi: 10.1097/ANC.0000000000000268.


Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that can be triggered by cytomegalovirus, a relatively common infectious exposure to neonates. The clinical presentation is common to many acute illnesses seen in extreme premature infants; however, there are key clinical and laboratory findings that can lead to the diagnosis.

Purpose: We present a case of an extreme premature infant of 25 weeks' gestation who developed cytomegalovirus-induced HLH. Using the current published protocols that are used in pediatric cancer can be adapted for use in a premature infant, which led to remission of HLH and eventual discharge from the neonatal intensive care unit.

Implications for practice: There are published treatment protocols used in pediatric oncology that when initiated early can lead to favorable outcomes and remission in even the most fragile neonates.

Implications for research: Additional studies are needed on the pharmacokinetics, dosing, and side effects on medications used for treatment of HLH in preterm infants. Additional research is needed to improve the clinician's ability to reach the diagnosis as well as define treatment strategies that provide optimal outcomes.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Agents, Phytogenic / therapeutic use*
  • Antiviral Agents / therapeutic use*
  • Bone Marrow / pathology
  • Cyclosporine / therapeutic use
  • Cytomegalovirus Infections / complications
  • Cytomegalovirus Infections / diagnosis
  • Cytomegalovirus Infections / drug therapy*
  • Dexamethasone / therapeutic use
  • Etoposide / therapeutic use
  • Ganciclovir / analogs & derivatives
  • Ganciclovir / therapeutic use
  • Glucocorticoids / therapeutic use*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Infant
  • Infant, Extremely Premature
  • Infant, Newborn
  • Killer Cells, Natural
  • Lymphohistiocytosis, Hemophagocytic / diagnosis
  • Lymphohistiocytosis, Hemophagocytic / drug therapy*
  • Lymphohistiocytosis, Hemophagocytic / etiology
  • Male
  • Receptors, Interleukin-2 / blood
  • Treatment Outcome
  • Valganciclovir


  • Antineoplastic Agents, Phytogenic
  • Antiviral Agents
  • Glucocorticoids
  • Immunosuppressive Agents
  • Receptors, Interleukin-2
  • Etoposide
  • Dexamethasone
  • Cyclosporine
  • Valganciclovir
  • Ganciclovir