Journal article
Erythrophagocytosis is not a reproducible finding in liver biopsies, and is not associated with clinical diagnosis of hemophagocytic lymphohistiocytosis
Virchows Archiv : an international journal of pathology, Vol.484(1), pp.61-69
01/2024
DOI: 10.1007/s00428-023-03683-7
PMID: 37924345
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease with high mortality. Liver involvement is common (based on elevated liver function tests) with most patients demonstrating acute hepatitis. Liver biopsies are frequently obtained in the setting of suspected HLH for the purpose of identification of erythrophagocytosis, and if present, this finding is thought to suggest or support the diagnosis of HLH. However, there are problems with this approach; in particular, we do not know whether this finding is reproducible or whether it is specific to HLH. Therefore, we conducted a multi-institutional study in which experienced liver pathologists reviewed images taken from liver biopsies from patients with normal liver, acute hepatitis, possible HLH, and clinical HLH to determine if there was agreement about the presence or absence of erythrophagocytosis, and to ascertain whether the finding corresponds to a clinical diagnosis of HLH. Twelve liver pathologists reviewed 141 images in isolation (i.e., no clinical information or diagnosis provided). These came from 32 patients (five normal, 17 acute hepatitis, six HLH, four possible HLH). The pathologists classified each image as negative, equivocal, or positive for erythrophagocytosis. Kappa was .08 (no agreement) for case-level and 0.1 for image-level (1.4% agreement, based on two images which were universally considered negative). There was no difference in the proportion of pathologists who diagnosed erythrophagocytosis among those with different diagnoses at case or image-level (p = 0.82 and p = 0.82, respectively). Thus, erythrophagocytosis is an entirely unreliable histologic parameter in liver, as it is irreproducible and not demonstrably associated with a clinical disease (namely, HLH). Unless and until more reliable guidelines can be established, pathologists should refrain from commenting on the presence or absence of erythrophagocytosis in liver biopsy.
Details
- Title: Subtitle
- Erythrophagocytosis is not a reproducible finding in liver biopsies, and is not associated with clinical diagnosis of hemophagocytic lymphohistiocytosis
- Creators
- Niyati Desai - Columbia University Irving Medical CenterSatoru Kudose - Columbia University Irving Medical CenterHelen E Remotti - Columbia University Irving Medical CenterArmando Del Portillo - Columbia University Irving Medical CenterLadan Fazlollahi - Columbia University Irving Medical CenterMichael J Lee - Columbia University Irving Medical CenterYuqing Xiong - Mass General BrighamRoger K Moreira - Mayo ClinicMarcela Salomao - Mayo Clinic in ArizonaMaria Isabel Fiel - Icahn School of Medicine at Mount SinaiRaul S Gonzalez - Emory University HospitalJoseph Misdraji - Yale UniversityRyan M Gill - University of California, San FranciscoJohn Hart - University of ChicagoDavid E Kleiner - National Cancer InstituteUta Drebber - University Hospital CologneAndrew M Bellizzi - University of IowaStephen M Lagana - Columbia University Irving Medical Center
- Resource Type
- Journal article
- Publication Details
- Virchows Archiv : an international journal of pathology, Vol.484(1), pp.61-69
- DOI
- 10.1007/s00428-023-03683-7
- PMID
- 37924345
- ISSN
- 0945-6317
- eISSN
- 1432-2307
- Grant note
- name: Intramural Research Program of the National Institutes of Health, National Cancer Institute.; name: The Young Investigator Grant of the National Kidney Foundation
- Language
- English
- Electronic publication date
- 11/04/2023
- Date published
- 01/2024
- Academic Unit
- Pathology
- Record Identifier
- 9984507026402771
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