Affiliation:
1. DNB, General Medicine
2. MD Path, DNB, Path
3. MD, DCH, MR CPCH, UK, FRCPCH UK
4. MD, General, Medicine
Abstract
Hemophagocytic lymphohistiocytosis is a hyper-inammatory condition that is either Familial (Primary)
or Secondary to autoimmune diseases , infection, malignancy or other triggers.It is a cytokine storm
syndrome where there inefcient antigen removal that leads to sustained cytokine release.It is a rare phenomenon occuring in
adults that has got a specic trigger which is less documented and have a good response to steroids where as Familial form is a
childhood disease due to genetic defects, both of which are life threatening and may need Allogenic bone marrow transplant.
Macrophage activation syndrome is also a subtype of this entity that occurs in the treatment phase of SLE and Still's disease.We
describe here 8 cases of secondary HLH, their primary triggers and treatment response.
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