Haemophagocytic Lymphohistiocytosis responding to withdrawal of gluten: a case report

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Title: Haemophagocytic Lymphohistiocytosis responding to withdrawal of gluten: a case report
Author(s): Fordham, NJ
Ajitsaria, R
Karnik, L
Chakravorty, S
Item Type: Journal Article
Abstract: Introduction This is the first documented case of a patient with Haemophagocytic Lymphohistiocytosis (HLH) in association with coeliac disease. There was complete clinical and biochemical remission of HLH following the introduction of a gluten free diet. Case presentation A seven-year old Caucasian girl presented with fevers, maculopapular rash with a recent history of tonsillitis. Blood tests revealed thrombocytopenia (64 x 109/L), anaemia (80g/L), hypofibrinogenaemia (1g/L) and hyperferritinaemia (71378µg/L). A bone marrow revealed evidence of haemophagocytosis, but tests for the genetic or familial associated HLH syndromes were negative. Screening tests for known secondary causes was negative. She was diagnosed with HLH, and following treatment with the HLH-2004 protocol these symptoms, in addition to the biochemical and haematological markers completely resolved. The patient presented again ten months later with fever, rash, and biochemical abnormalities suggestive of HLH. Tissue transglutamase was markedly raised and blood tests revealed a genetic susceptibly to coeliac disease in the form of HLA DQ2 positivity. She commenced a gluten free diet and there was complete symptomatic and biochemical response without any further chemotherapy. She had further episodic rashes, each associated with the accidental intake of gluten. Conclusion This is to our knowledge the first documented case of HLH in association with coeliac disease. There was no other secondary cause found; she initially responded to chemo-immunotherapy specific for HLH but relapsed within a few months of cessation of treatment and then achieved complete remission on gluten withdrawal alone.
Publication Date: 22-Sep-2016
Date of Acceptance: 25-Aug-2016
URI: http://hdl.handle.net/10044/1/39515
DOI: https://dx.doi.org/10.1186/s13256-016-1049-6
ISSN: 1752-1947
Publisher: BioMed Central
Journal / Book Title: Journal of Medical Case Reports
Volume: 10
Copyright Statement: © 2016 The Author(s). Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. 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.
Keywords: General & Internal Medicine
1199 Other Medical And Health Sciences
Publication Status: Published
Article Number: 262
Appears in Collections:Faculty of Medicine

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