Hematological malignancies in systemic lupus erythematosus: clinical characteristics, risk factors, and prognosis—a case-control study

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Abstract

Background

Systemic lupus erythematosus (SLE) is a chronic and complex multi-system autoimmune disorder. Higher risks of hematological malignancies (HM) were observed in SLE patients, which was associated with higher mortality. The mechanism and risk factors of HM oncogenesis in SLE patients are still under investigation. The aim of this study was to explore clinical characteristics, risk factors, and prognosis of SLE patients with or without HM in the Chinese population.

Methods

A retrospective, case-controlled study was conducted in 72 SLE patients between January 2013 and December 2020. Clinical and laboratory data were collected and compared between the two groups of patients with HM and those without HM. Logistic regression analysis was performed to determine risk factors of HM oncogenesis. The survival rate was estimated by Kaplan-Meier methods and Cox proportional hazards regression analysis.

Results

Among 72 SLE patients in this study, fifteen complicated with HM and 57 without HM were identified. The incidence rate of HM was approximately 0.24% with elevated standardized incidence ratios of lymphoma and leukemia (27.559 and 12.708, respectively). Patients with HM were older when diagnosed with SLE, with a higher frequency of infection and splenomegaly, lower levels of hemoglobin and high-density lipoprotein compared with those without HM. Fewer patients with HM expressed positive anti-dsDNA antibody (26.7% vs 66.7%, P = 0.005) or received hydroxychloroquine treatment (40.0% vs 86.0%, P = 0.001). Older age at SLE diagnosis ( OR=1.122, 95% CI: 1.037–1.214) was regarded as an independent risk factor of HM oncogenesis. Female ( RR= 0.219, 95% CI: 0.070–0.681) and hydroxychloroquine ( RR= 0.281, 95% CI: 0.094–0.845) were protective factors of mortality in SLE patients.

Conclusions

SLE patients with an older age are at an increased risk of HM carcinogenesis. The prognosis of male patients with SLE tends to be poorer whether complicated with HM. The association of antinuclear antibody spectrum, medication, and HM oncogenesis in SLE needs further investigation.

Supplementary Information

The online version contains supplementary material available at 10.1186/s13075-021-02692-8.

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Most cited references 55

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The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new knowledge regarding the immunology of SLE. The classification criteria were derived from a set of 702 expert-rated patient scenarios. Recursive partitioning was used to derive an initial rule that was simplified and refined based on SLICC physician consensus. The SLICC group validated the classification criteria in a new validation sample of 690 new expert-rated patient scenarios. Seventeen criteria were identified. In the derivation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (49 versus 70; P = 0.0082) and had greater sensitivity (94% versus 86%; P < 0.0001) and equal specificity (92% versus 93%; P = 0.39). In the validation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (62 versus 74; P = 0.24) and had greater sensitivity (97% versus 83%; P < 0.0001) but lower specificity (84% versus 96%; P < 0.0001). The new SLICC classification criteria performed well in a large set of patient scenarios rated by experts. According to the SLICC rule for the classification of SLE, the patient must satisfy at least 4 criteria, including at least one clinical criterion and one immunologic criterion OR the patient must have biopsy-proven lupus nephritis in the presence of antinuclear antibodies or anti-double-stranded DNA antibodies. Copyright © 2012 by the American College of Rheumatology.

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The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes.

James W Vardiman, Juergen Thiele, Daniel Arber … (2009)

Recently the World Health Organization (WHO), in collaboration with the European Association for Haematopathology and the Society for Hematopathology, published a revised and updated edition of the WHO Classification of Tumors of the Hematopoietic and Lymphoid Tissues. The 4th edition of the WHO classification incorporates new information that has emerged from scientific and clinical studies in the interval since the publication of the 3rd edition in 2001, and includes new criteria for the recognition of some previously described neoplasms as well as clarification and refinement of the defining criteria for others. It also adds entities-some defined principally by genetic features-that have only recently been characterized. In this paper, the classification of myeloid neoplasms and acute leukemia is highlighted with the aim of familiarizing hematologists, clinical scientists, and hematopathologists not only with the major changes in the classification but also with the rationale for those changes.

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Author and article information

Contributors

Shengyun Liu:

ORCID: http://orcid.org/0000-0002-0426-1705

fccliusy2@zzu.edu.cn

Journal

Journal ID (nlm-ta): Arthritis Res Ther

Journal ID (iso-abbrev): Arthritis Res Ther

Title: Arthritis Research & Therapy

Publisher: BioMed Central (London )

ISSN (Print): 1478-6354

ISSN (Electronic): 1478-6362

Publication date (Electronic): 3 January 2022

Publication date PMC-release: 3 January 2022

Publication date (Print): 2022

Volume: 24

Electronic Location Identifier: 5

Affiliations

GRID grid.412633.1, Department of Rheumatology and Immunology, , the First Affiliated Hospital of Zhengzhou University, ; No.1 Jianshe East Road, Zhengzhou, 450052 Henan Province China

Author information

Shengyun Liu http://orcid.org/0000-0002-0426-1705

Article

Publisher ID: 2692

DOI: 10.1186/s13075-021-02692-8

PMC ID: 8722144

PubMed ID: 34980230

SO-VID: bf9be272-818c-4269-b0a9-9ac446f252c9

License:

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

History

Date received : 9 March 2021

Date accepted : 5 December 2021

Funding

Funded by: FundRef http://dx.doi.org/10.13039/501100011447, Science and Technology Department of Henan Province;

Award ID: SB201901020

Award Recipient : Shengyun Liu

Funded by: FundRef http://dx.doi.org/10.13039/501100013057, Innovation Scientists and Technicians Troop Construction Projects of Henan Province;

Award ID: SBGJ202003024

Award Recipient : Jinyan Guo

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ScienceOpen disciplines: Orthopedics

Keywords: systemic lupus erythematosus,hematological malignancies,risk factors,survival,prognosis

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ScienceOpen disciplines: Orthopedics

Keywords: systemic lupus erythematosus, hematological malignancies, risk factors, survival, prognosis

Hematological malignancies in systemic lupus erythematosus: clinical characteristics, risk factors, and prognosis—a case-control study

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Most cited references 55

Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus.

Systemic lupus erythematosus disease activity index 2000.

The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes.

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