Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Aug 11;15(8):e0237530.
doi: 10.1371/journal.pone.0237530. eCollection 2020.

Identifying acute lymphoblastic leukemia mimicking juvenile idiopathic arthritis in children

Ninna Brix  1   2 , Steen Rosthøj  2 , Mia Glerup  1 , Henrik Hasle  1 , Troels Herlin  1
Affiliations

Identifying acute lymphoblastic leukemia mimicking juvenile idiopathic arthritis in children

Ninna Brix et al. PLoS One. .

Abstract

Objective: Acute lymphoblastic leukemia (ALL) may present with arthritis implying the risk of being misdiagnosed as juvenile idiopathic arthritis (JIA). The aim of this study was to identify predictors for ALL based on clinical and laboratory information.

Methods: This cross-sectional, retrospective study compared clinical presentation and laboratory results of 26 children with ALL and arthritis versus 485 children with JIA (433 non-systemic, 52 systemic JIA). Using a Bayesian score approach the findings were evaluated by calculating odds ratios (OR) and lnOR as a measure of diagnostic weight.

Results: Distinction on clinical grounds was difficult, as even a high number of joints involved did not exclude ALL. One or more hematologic cell counts were low (Hb <10 g/dL, platelet count <100 x 109/L, neutrophil count < 1.0 x 109/L) in 92% with ALL, 25% with systemic JIA and 10% with non-systemic JIA. Neutropenia and thrombocytopenia had the highest ORs of 128 (95% CI 43-387) and 129 (95% CI 26-638), each giving a diagnostic weight of 4. The estimated risks of ALL were 0.2% with normal cell counts and 9%, 67% and 100% when one, two or three cell lines were affected.

Conclusion: A simple count of cell lines with low counts can serve as a basic diagnostic strategy. Children with tri- or bilinear involvement should be referred to a bone marrow, and those with unilinear involvement a thorough screen for further evidence of ALL (organomegaly, ESR, LDH, uric acid, and blood smear).

PubMed Disclaimer

Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Fig 1
Fig 1
Box plot comparing laboratory values as median (horizontal line), interquartile range (the box) and total range (vertical line with spots showing outliners) for children with ALL, systemic JIA and non-systemic JIA. * p-value <0.001 ** p = 0.005.
See this image and copyright information in PMC

References

    1. Brix N, Rosthøj S, Herlin T, Hasle H. Arthritis as presenting manifestation of acute lymphoblastic leukaemia in children. Arch Dis Child. 2015;100(9):821–5. 10.1136/archdischild-2014-307751 - DOI - PubMed
    1. Riise Ø, Handeland K, Cvancarova M, Wathne KO, Nakstad B, Flatø B et al. Incidence and characteristics of arthritis on Norweigan children: a population-based study. Pediatrics. 2008;121(299–306):e. - PubMed
    1. Berntson L, Andersson Gäre B, Fasth A, Herlin T, Kristinsson J, Nordic study group et al. Incidence of juvenile idiopathic arthritis in the Nordic countries. A population based study with special reference to the validity og the ILAR and EULAR criteria. J Rheumatol. 2003;30:2271–82. - PubMed
    1. Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet. 2007;369(9563):767–78. 10.1016/S0140-6736(07)60363-8 - DOI - PubMed
    1. Marwaha R, Kulkarn K, Bansal D, Trehan A. Acute lymphoblastic leukemia masquerading as juvenile rheumatoid arthritis: diagnostic pitfall and association with survival. Ann Hematol. 2010;89(3):249–54. 10.1007/s00277-009-0826-3 - DOI - PubMed

Publication types