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Cited by 130Autoimmune and Inflammatory Disorders ResearchKawasaki Disease and Coronary ComplicationsImmunodeficiency and Autoimmune Disorders

The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis

Gupta, Abha, Tyrrell, Pascal, Valani, Rahim, Benseler, Susanne, Weitzman, Sheila, Abdelhaleem, Mohamed·2008·Pediatric Blood & Cancer
DOI 10.1002/pbc.21564
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Abstract

Background: The identification of hemophagocytosis (HPC) in tissue or bone marrow (BM) represents only one of 5/8 criteria needed for the diagnosis of hemophagocytic lymphohistiocytosis (HLH). Yet, confirmation of HPC in bone marrow aspirates (BMA) is often relied upon to make therapeutic decisions. There is no standardized reporting criteria for the definition of “positive” BMA, and likely differs between institutions. The purpose of this study was to quantify the number of HPC in the initial BMA in patients diagnosed with HLH at our institution. Procedure: Patient charts were retrospectively reviewed. Numbers of HPC were counted per 500 nucleated cells in initial BMA. Results: Fifty‐eight percent of patients had at least one HPC per 500 nucleated cells. Median number of HPC per 500 cells was 1 (0–12). Median time from initial BMA to HLH diagnosis was 0 days (−3 to 11), suggesting that HLH diagnosis was made regardless of the results of this initial BMA. Conclusion: The number of HPC at initial BMA is often low and variable, confirming that a BMA lacking HPC does not rule out the diagnosis of HLH, and a negative initial BMA should not delay therapy. We recommend that the BMA report should document negative as well as any positive findings of HPC. Pediatr Blood Cancer 2008;51:402–404. © 2008 Wiley‐Liss, Inc.

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  • Over 40% of patients with MAS/HLH do not have hemophagocytes in bone marrow aspirates.

    “Although sampling error is always of concern, it has been reported that over 40% of patients with MAS/HLH do not have hemophagocytes in bone marrow aspirates, suggesting a separation of disease initiation from the development of hemophagocytes (23).”

    From Repeated TLR9 stimulation results in macrophage activation syndrome–like disease in mice (2011) · cites this paper as [23]

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Authors
Gupta, A., Tyrrell, P., Valani, R., Benseler, S., Weitzman, S., & Abdelhaleem, M.
Affiliations
Hospital for Sick Children (CA)
Corresponding author
Gupta, Abha
ORCID
Venue
Pediatric Blood & Cancer
Publisher
Wiley
Published
2008-06-03
Volume
51
Issue
3
Pages
402-404
ISSN
  • 1545-5009 (ISSN-L)
  • 1545-5017
DOI
10.1002/pbc.21564
OpenAlex
W2012471701
Cited by
130
References
14
Citations by year
2026: 3 · 2025: 4 · 2024: 6 · 2023: 2 · 2022: 5 · 2021: 7 · 2020: 5 · 2019: 12 · 2018: 13 · 2017: 7 · 2016: 7 · 2015: 10 · 2014: 17 · 2013: 6 · 2012: 13
FWCI
2.81
Type
journal article
Language
en
Open access
closed
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations
  • Pediatric Blood & Cancer (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Hematology › Autoimmune and Inflammatory Disorders Research
  • Health Sciences › Medicine › Surgery › Kawasaki Disease and Coronary Complications
  • Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
Keywords
hemophagocytic lymphohistiocytosis, hemophagocytosis, bone marrow aspirate, diagnostic criteria, reporting criteria, delayed diagnosis, pediatric patients
MeSH terms
Adolescent, Biopsy, Needle, Bone Marrow Examination, Cell Count, Child, Child, Preschool, Diagnostic Errors, Humans, Infant, Retrospective Studies, Observer Variation, Practice Guidelines as Topic, Lymphohistiocytosis, Hemophagocytic

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