Serum neopterin levels as a diagnostic marker of hemophagocytic lymphohistiocytosis syndrome

Maria F. Ibarra, Marisa Klein-Gitelman, Elaine Morgan, Maria Proytcheva, Christine Sullivan, Gabrielle Morgan, Lauren M. Pachman, Maurice R G O'Gorman

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

The objective of this study was to retrospectively evaluate the utility of serum neopterin as a diagnostic marker of hemophagocytic lymphohistiocytosis (HLH). The medical records of patients diagnosed with HLH (familial and secondary) between January 2000 and May 2009 were reviewed retrospectively, and clinical and laboratory information related to HLH criteria, in addition to neopterin levels, was recorded. A group of 50 patients with active juvenile dermatomyositis (JDM) (who routinely have neopterin levels assessed) served as controls for the assessment of the accuracy, sensitivity, and specificity of neopterin as a diagnostic test for HLH. The Pearson correlation was used to measure the association between serum neopterin levels and established HLH-related laboratory data. Serum neopterin levels were measured using a competitive enzyme immunoassay. During the time frame of the study, 3 patients with familial HLH and 18 patients with secondary HLH were identified as having had serum neopterin measured (all HLH patients were grouped together). The mean neopterin levels were 84.9 nmol/liter (standard deviation [SD], 83.4 nmol/liter) for patients with HLH and 21.5 nmol/liter (SD, 10.13 nmol/liter) for patients with JDM. A cutoff value of 38.9 nmol/liter was 70% sensitive and 95% specific for HLH. For HLH patients, neopterin levels correlated significantly with ferritin levels (r = 0.76, P = 0.0007). In comparison to the level in a control group of JDM patients, elevated serum neopterin was a sensitive and specific marker for HLH. Serum neopterin has value as a diagnostic marker of HLH, and prospective studies are under way to further evaluate its role as a marker for early diagnosis and management of patients.

Original languageEnglish (US)
Pages (from-to)609-614
Number of pages6
JournalClinical and Vaccine Immunology
Volume18
Issue number4
DOIs
StatePublished - Apr 1 2011

Fingerprint

Neopterin
Hemophagocytic Lymphohistiocytosis
Serum
Ferritins
Immunoenzyme Techniques
Routine Diagnostic Tests
Medical Records

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Clinical Biochemistry
  • Microbiology (medical)

Cite this

Ibarra, Maria F. ; Klein-Gitelman, Marisa ; Morgan, Elaine ; Proytcheva, Maria ; Sullivan, Christine ; Morgan, Gabrielle ; Pachman, Lauren M. ; O'Gorman, Maurice R G. / Serum neopterin levels as a diagnostic marker of hemophagocytic lymphohistiocytosis syndrome. In: Clinical and Vaccine Immunology. 2011 ; Vol. 18, No. 4. pp. 609-614.
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Serum neopterin levels as a diagnostic marker of hemophagocytic lymphohistiocytosis syndrome. / Ibarra, Maria F.; Klein-Gitelman, Marisa; Morgan, Elaine; Proytcheva, Maria; Sullivan, Christine; Morgan, Gabrielle; Pachman, Lauren M.; O'Gorman, Maurice R G.

In: Clinical and Vaccine Immunology, Vol. 18, No. 4, 01.04.2011, p. 609-614.

Research output: Contribution to journalArticle

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