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Haemophagocytic lymphohistiocytosis in pregnancy

Bethan Goulden, Taryn Youngstein, Ian Giles, Jessica J Manson and David Williams
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DOI: https://doi.org/10.7861/clinmed.Let.21.6.2
Clin Med November 2021
Bethan Goulden
Rheumatology and general internal medicine registrar, University College London Hospital, London, UK
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Taryn Youngstein
Consultant rheumatologist, Hammersmith Hospital, London, UK and honorary senior lecturer, National Heart and Lung Institute, London, UK
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Ian Giles
Professor of rheumatology, UCL Centre for Rheumatology, London, UK and honorary consultant rheumatologist, University College London Hospital, London, UK
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Jessica J Manson
Consultant rheumatologist, University College London Hospital, London, UK, associate professor, University College London, London, UK and co-chair, Hyperinflammation and HLH Across Speciality Collaboration, London, UK
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David Williams
Consultant obstetric physician UCL EGA Institute for Women's Health, London, UK
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Editor - The annual confidential enquiries into maternal mortality in the UK, published by MBBRACE, state that we should ‘Treat pregnant and postpartum women the same as non-pregnant women unless there is a clear reason not to’.1 Consecutive MBRRACE reports include numerous maternal deaths occurring after standard of care treatment was withheld or delayed due to concerns about its safety in pregnancy. In the paper by Jha et al, the authors state that ‘the safety and efficacy of ... cyclosporin, rituximab and immunoglobulin during pregnancy and lactation are yet to be established.2 Evidence-based statements supporting the use in pregnancy of cyclosporin, intravenous immunoglobulins and, for life-threatening maternal disease, rituximab have been produced by the American College of Rheumatology, European League Against Rheumatism and British Society for Rheumatology.3–5 Therefore, these treatments should not be withheld from pregnant women due to concerns about fetal harm. This is particularly important for women with severe maternal disease such as haemophagocytic lymphohistiocytosis (HLH), as untreated disease could be harmful to both mother and baby.

The HLH label should be viewed as we see sepsis, as a starting point to initiate management while striving to identify an underlying source/trigger. The immunological changes of pregnancy can trigger HLH, with delivery being curative, but HLH in pregnancy can also occur secondary to infection, autoimmunity/autoinflammation and haematological malignancy.6 Jha et al state that ‘Biopsy of liver, spleen or lymph nodes could not have revealed any extra information to change the diagnosis’, but we would advise caution with this assumption.2 Steroids are often employed early in HLH to control hyperinflammation but can mask or delay the identification of triggers such as lymphoma.7 Our practice is to undertake repeated histological examination of abnormal organs, lymph nodes and bone marrow alongside the use of steroid sparing agents, where possible. Managing a pregnant woman with HLH is best done within a multidisciplinary team (MDT) setting, including rheumatology, haematology, infectious diseases, obstetrics and obstetric medicine and with advice from an HLH MDT, in order to best judge the timing of investigations and delivery.

Conflicts of interest

Taryn Youngstein is on industry-sponsored advisory boards for Sobi, Novartis and Roche. Ian Giles is a speaker, has advisory board fees and an unrestricted research grant from UCB Pharma.

  • © Royal College of Physicians 2021. All rights reserved.

References

    1. MBRRACE-UK
    . Confidential Enquiry into Maternal Deaths. NPEU. www.npeu.ox.ac.uk/mbrrace-uk/reports/confidential-enquiry-into-maternal-deaths [Accessed on 14 September 2021].
  1. ↵
    1. Jha N
    , Balachandran DM Jha AK. Fever, cough and gastrointestinal symptoms in a pregnant woman. Clin Med 2021;21:e526–8.
    OpenUrlAbstract/FREE Full Text
  2. ↵
    1. Flint J
    , Panchal S Hurrell A, et al. BSR and BHPR guideline on prescribing drugs in pregnancy and breastfeeding - Part I: standard and biologic disease modifying anti-rheumatic drugs and corticosteroids. Rheumatology 2016;55:1693–7.
    OpenUrlCrossRefPubMed
  3. ↵
    1. Skorpen CG
    , Hoeltzenbein M Tincani A, et al. The EULAR points to consider for use of antirheumatic drugs before pregnancy, and during pregnancy and lactation. Ann Rheum Dis 2016;75:795–810.
    OpenUrlAbstract/FREE Full Text
  4. ↵
    1. Sammaritano LR
    , Bermas BL, Chakravarty EE, et al. 2020 American College of Rheumatology Guideline for the Management of Reproductive Health in Rheumatic and Musculoskeletal Diseases. Arthritis Care Res (Hoboken) 2020;72:461–88.
    OpenUrl
  5. ↵
    1. Wilson-Morkeh H
    , Frise C Youngstein T. Haemophagocytic lymphohistiocytosis in pregnancy. Obstet Med 2021:1753495X211011913.
  6. ↵
    1. Hutchinson M
    , Tattersall RS Manson JJ. Haemophagocytic lymphohisticytosis - an underrecognized hyperinflammatory syndrome. Rheumatology 2019;58(Supplement 6):vi23–30.
    OpenUrlPubMed
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Haemophagocytic lymphohistiocytosis in pregnancy
Bethan Goulden, Taryn Youngstein, Ian Giles, Jessica J Manson, David Williams
Clinical Medicine Nov 2021, 21 (6) e682-e683; DOI: 10.7861/clinmed.Let.21.6.2

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Haemophagocytic lymphohistiocytosis in pregnancy
Bethan Goulden, Taryn Youngstein, Ian Giles, Jessica J Manson, David Williams
Clinical Medicine Nov 2021, 21 (6) e682-e683; DOI: 10.7861/clinmed.Let.21.6.2
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