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Chicago Ridge, IL, United States

Ibarra M.F.,Childrens Memorial Hospital | Ibarra M.F.,Northwestern University | Klein-Gitelman M.,Childrens Memorial Hospital | Klein-Gitelman M.,Northwestern University | And 10 more authors.
Clinical and Vaccine Immunology | Year: 2011

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. Copyright © 2011, American Society for Microbiology. All Rights Reserved.


Wahezi D.M.,Childrens Hospital at Montefiore | Ilowite N.T.,Childrens Hospital at Montefiore | Rajpathak S.,Childrens Hospital at Montefiore | Rand J.H.,Childrens Hospital at Montefiore | Rand J.H.,Hematology Laboratories
Journal of Rheumatology | Year: 2012

Objective. The underlying mechanism(s) by which antiphospholipid antibodies (aPL) result in thrombosis remains poorly understood. A significant body of evidence has evolved to support the hypothesis that antibody-mediated disruption of an annexin A5 anticoagulant shield may play a role in the pathogenesis; this proposed mechanism has not been previously studied in children. Methods. We investigated the association between aPL and resistance to annexin A5 anticoagulant activity in 90 children with a variety of rheumatic diseases using a novel mechanistic assay, the annexin A5 resistance assay (A5R). Results. Patients with a diagnosis of primary aPL syndrome, systemic lupus erythematosus, and mixed connective tissue disease demonstrated lower mean A5R levels (p = 0.030), higher prevalence of positive aPL (p < 0.001), and more thrombotic events (p = 0.014) compared to those with other diagnoses. Patients with persistently positive aPL had significantly lower mean A5R compared to patients with no aPL (mean A5R = 203% ± 44% vs 247% ± 35%; p < 0.001), whereas patients with transient aPL did not. Patients with thrombosis had lower A5R levels compared to those without thrombosis (mean A5R = 207% ± 36% vs 237% ± 46%; p = 0.048). Conclusion. Children and adolescents with rheumatic diseases and persistent aPL have reduced annexin A5 anticoagulant activity, whereas transient, nonpathogenic aPL have less effect on annexin A5 activity. The Journal of Rheumatology Copyright © 2012. All rights reserved.


Wang Q.,Peking University | Liu H.,Hematology Laboratories | Zhang X.,Hematology Laboratories | Liu Q.,Peking University | And 4 more authors.
Blood | Year: 2010

Donor lymphocyte infusion is an alternative treatment for Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (LPDs) but with risk of graft-versushost diseases (GVHDs). According to the fetal-maternal microchimerism tolerance, we assumed that maternal lymphocyte infusion may be effective without causing GVHD. In 54 cases when a child required cytotherapy or hematopoietic stem cell transplantation, we studied the mother for child-mother microchimerism with use of insertion-deletion polymorphisms as allogeneic markers and a combination of nested polymerase chain reaction (PCR) and real-time quantitative PCR. Thirteen mothers were child-microchimerismpositive at the ratio of 10-5-10-3. Among them, 5 children had non-transplantassociated, EBV+ T-cell LPD. In these 5 cases, high doses of human leukocyte antigen-haploidentical maternal peripheral blood mononuclear cells (> 108/kg/infusion) were infused 1-4 times. Symptoms of all 5 patients improved between 3 and 10 days after the infusion; thereafter, 3 cases showed complete remission for 6-18 months without further therapy and 2 had partial remission. During the period of observation, none developed obvious GVHD. By quantitative PCR, in some patients maternal cells were found to be eliminated or decreased after infusions, indicating existence of host-versusgraft reaction. We suggest that high doses of mother's lymphocyte infusion may be an effective and safe treatment for nontransplant-associated EBV+ T-cell LPD. © 2010 by The American Society of Hematology.

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