Imperial College London
Alternate

DrSubarnaChakravorty

Faculty of Medicine, Department of Immunology and Inflammation

Honorary Clinical Senior Lecturer
 
 
 
//

Contact

 

+44 (0)20 3299 3773s.chakravorty

 
 
//

Location

 

Medical SchoolSt Mary's Campus

//

Summary

 

Publications

Citation

BibTex format

@article{Fordham:2016:10.1186/s13256-016-1049-6,
author = {Fordham, NJ and Ajitsaria, R and Karnik, L and Chakravorty, S},
doi = {10.1186/s13256-016-1049-6},
journal = {Journal of Medical Case Reports},
title = {Haemophagocytic Lymphohistiocytosis responding to withdrawal of gluten: a case report},
url = {http://dx.doi.org/10.1186/s13256-016-1049-6},
volume = {10},
year = {2016}
}
Download

RIS format (EndNote, RefMan)

TY  - JOUR
AB  - IntroductionThis 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 presentationA 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. ConclusionThis 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.
AU  - Fordham,NJ
AU  - Ajitsaria,R
AU  - Karnik,L
AU  - Chakravorty,S
DO  - 10.1186/s13256-016-1049-6
PY  - 2016///
SN  - 1752-1947
TI  - Haemophagocytic Lymphohistiocytosis responding to withdrawal of gluten: a case report
T2  - Journal of Medical Case Reports
UR  - http://dx.doi.org/10.1186/s13256-016-1049-6
UR  - http://hdl.handle.net/10044/1/39515
VL  - 10
ER  -
Download