Marrow Assessment for Hemophagocytic Lymphohistiocytosis Demonstrates Poor Correlation With Disease Probability
ABSTRACT Objectives: To evaluate the amount of hemophagocytosis in 64 marrow core biopsy specimens and aspirates from 58 patients with clinical suspicion for secondary hemophagocytic lymphohistiocytosis (HLH) or reported findings of hemophagocytosis. Methods: A review of medical records assigned patients to a low-risk (45 patients) or high-risk (13 patients) HLH group, and association with histologic findings was examined using the Fisher exact test. Results: The amount of hemophagocytosis in aspirate or the core biopsy specimen did not correlate with disease probability (P = .17 and P = .63, respectively). Of the clinical/laboratory criteria assessed, the most significant correlations with HLH were highly elevated ferritin (P = .01), cytopenias (P = .02), and fever (P = .009). Conclusions: Our findings indicated that marrow histologic findings alone do not reliably predict the probability of HLH, and an isolated finding of hemophagocytosis, even when present in a high amount, lacks specificity for HLH.
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ABSTRACT: Objective. To compare the capacity of hemophagocytic lymphohistiocytosis (HLH)-2004 diagnostic guidelines and preliminary diagnostic guidelines for systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS) to discriminate MAS complicating sJIA from two potentially confusable conditions, represented by active sJIA without MAS and systemic infection. Methods. International pediatric rheumatologists and hemato-oncologists were asked to collect retrospectively the clinical information of patients with sJIA-associated MAS and confusable conditions. The ability of guidelines to differentiate MAS from control diseases was evaluated by calculating sensitivity and specificity and with kappa statistics. Owing to unavailability of assessment of bone marrow hemophagocytosis, NK cell activity and soluble CD25 levels, HLH-2004 guidelines were adapted to enable the diagnosis of MAS when 3/5 or 4/5 of the remaining items were present. Results. The study sample included 362 patients with sJIA and MAS, 404 patients with active sJIA without MAS, and 345 patients with systemic infection. The best capacity to differentiate MAS from sJIA without MAS was shown by the preliminary MAS guidelines. The adapted 3/5 HLH-2004 guidelines performed better than the adapted 4/5 guidelines in distinguishing MAS from active sJIA without MAS. The adapted 3/5 HLH-2004 guidelines and preliminary MAS guidelines with the addition of ferritin ≥ 500 ng/mL discriminated best between MAS and systemic infections. Conclusion. Preliminary MAS guidelines showed the strongest ability to identify MAS in sJIA. The addition of hyperferritinemia enhanced their capacity to differentiate MAS from systemic infections. HLH-2004 guidelines are likely not appropriate for identification of MAS in children with sJIA. © 2014 American College of Rheumatology.
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ABSTRACT: Macrophage activation syndrome is the rheumatic disease-associated member of a group of hyperinflammatory syndromes characterized by uncontrolled cytokine storm. In this review, we highlight recent publications related to the pathoetiology of hyperinflammatory syndromes with an emphasis on how this new knowledge will guide our diagnosis, treatment, and future research efforts to better understand these deadly conditions.Current Opinion in Rheumatology 07/2014; DOI:10.1097/BOR.0000000000000093 · 5.07 Impact Factor
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ABSTRACT: Objectives Bone marrow (BM) aspiration and trephine biopsy is one of the most valuable procedures in the evaluation of hematological disorders. There is a shortage of published literature regarding the indications, procedure, and outcome of bone marrow examination (BME) in neonates and infants. The aim of the present study is to analyze the common indications of performing BME and to assess the spectrum of disorders diagnosed from BM of neonates and infants. Methods A retrospective analysis of BMEs performed in infants over a period of 5 years, between 2009 and 2013 was done. Results and discussion A total of 297 BME were performed on 285 infants, which constitutes 10.3% of pediatric BME procedures during the same period. In our institute, BME is routinely performed by trained pathologists from posterior superior iliac spine in children including infants and neonates with an overall sample adequacy of 97%. Evaluation of cytopenias and suspicion of storage disorder were the most common indications for BME procedure, while acute leukemias and storage disorders were the most common diagnoses offered in infant BM. Conclusions Posterior superior iliac spine is a good site of BME in neonates and infants. BM trephine biopsy is a difficult procedure in this age group, however remains indispensable in situations where an infiltrative pathology is suspected. BME not only helps to make specific diagnoses but should also be used as an extremely valuable, quick, and economically viable procedure to exclude major hematological disorders including certain forms of storage disorder and hematological malignancy in this age group.Hematology (Amsterdam, Netherlands) 08/2014; 20(3). DOI:10.1179/1607845414Y.0000000184 · 1.19 Impact Factor