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1/ What are your thoughts when you get consulted about HLH?
Scared?
Puzzled?
Maybe even excited?
Well, @freedobaggins led an awesome #HemeFridays conference on the topic
Starting off with a very useful paper for all things HLH:
https://ashpublications.org/blood/article/133/23/2465/273833/Recommendations-for-the-management-of
Scared? Puzzled? Maybe even excited?Well, @freedobaggins led an awesome #HemeFridays conference on the topic
Starting off with a very useful paper for all things HLH:
https://ashpublications.org/blood/article/133/23/2465/273833/Recommendations-for-the-management-of
2/ Pathophysiology
IFN-Ī³ is a primary mediator of HLH.
How do we know this? Well patients given IFN-Ī³ get all the signs and symptoms, and itās linked to most of the findings.
Hereās an awesome graphic, again by @freedobaggins:
IFN-Ī³ is a primary mediator of HLH.
How do we know this? Well patients given IFN-Ī³ get all the signs and symptoms, and itās linked to most of the findings.
Hereās an awesome graphic, again by @freedobaggins:
3/ Diagnosis
How do you have a
standard for a human construct of a disease?
Any tools are inherently biased and you canāt think about this like you do a disease with clean pathophysiology.
Tools like H-score are helpful though! https://www.mdcalc.com/hscore-reactive-hemophagocytic-syndrome
How do you have a
standard for a human construct of a disease?Any tools are inherently biased and you canāt think about this like you do a disease with clean pathophysiology.
Tools like H-score are helpful though! https://www.mdcalc.com/hscore-reactive-hemophagocytic-syndrome
4/
While soluble IL-2R is probably the best marker of HLH, it is a non-specific marker of T-cell activation ā not specific.
Also important to know that IL-2R is NOT pathogenic (i.e. does not mediate disease).
While soluble IL-2R is probably the best marker of HLH, it is a non-specific marker of T-cell activation ā not specific.
Also important to know that IL-2R is NOT pathogenic (i.e. does not mediate disease).
5/ Treatment
Mainstay of tx is dexamethasone
Cyclosporine & Etoposide commonly used as anti-T cell drugs
Empalumab = mAb against IFN-Ī³, studied in kids with primary HLH
Mainstay of tx is dexamethasoneCyclosporine & Etoposide commonly used as anti-T cell drugsEmpalumab = mAb against IFN-Ī³, studied in kids with primary HLH
6/ Terminology
HLH is a syndrome characterized by excessive inflammatory response
Macrophage activation syndrome or hemophagocytic syndrome are older terms for HLH occurring in pts with an underlying autoimmune disease
Kids often 1ā, Adults are 2ā
HLH is a syndrome characterized by excessive inflammatory response Macrophage activation syndrome or hemophagocytic syndrome are older terms for HLH occurring in pts with an underlying autoimmune diseaseKids often 1ā, Adults are 2ā
7/ Letās explore a few representative cases help to show the complexity of this diagnosis!
8/
A patient in their 60s presents in with fevers, transaminitis, hypotension, negative culture. H score shows >99% chance of HLH.
Do you pursue bone marrow?
A patient in their 60s presents in with fevers, transaminitis, hypotension, negative culture. H score shows >99% chance of HLH.
Do you pursue bone marrow?
9/
Even when the diagnosis is certain, it is VITAL to know the primary disease; tx starts with tx of primary process. Hematologic malignancies are a common primary disease.
Learning point: If you have even a slight suspicion of HLH, do the marrow day 1!
Even when the diagnosis is certain, it is VITAL to know the primary disease; tx starts with tx of primary process. Hematologic malignancies are a common primary disease.
Learning point: If you have even a slight suspicion of HLH, do the marrow day 1!
10/
A patient in their 40s p/w fevers, night sweats, splenomegaly. They have a normal BMBx at another hospital prior to transfer. H score is low and she is treated for adult-onset Stillās.
But then she worsened and review of marrow (and sol IL2R which had returned) c/w HLH.
A patient in their 40s p/w fevers, night sweats, splenomegaly. They have a normal BMBx at another hospital prior to transfer. H score is low and she is treated for adult-onset Stillās.
But then she worsened and review of marrow (and sol IL2R which had returned) c/w HLH.
11/ So the next time you are consulted with the question āIs this HLH?ā consider this timeline schema.
12/ How do you all think about HLH?
Do you find the H-score helpful?
What's still confusing about the diagnosis or management?
Do you find the H-score helpful? What's still confusing about the diagnosis or management?
