[Adult-hlh-mas] Possible HLH Case Parkland [securemail]
Bonnie.Prokesch at UTSouthwestern.edu
Mon Aug 6 09:51:12 CDT 2018
Thanks for posting! This appears to be a complicated case. His CD4 count is 13 and it appears that he is likely connected with HIV and Hep B. Agree with Chris that generally speaking if the inflammatory response is driven by histo (which in and of itself can cause high ferritin), amphotericin should calm things down a bit. That being said, it seems as though the infectious work up is still in process (he clearly has HIV, syphilis, histo, and perhaps TB and or dMAC). Thus, I do not think I would start much else treatment-wise for HLH at present. I would recommend lymph node or BM biopsy before we increase his steroids further (I saw that there has been discussion already regarding a BM biopsy). I think the marrow should be sent for path as well as culture (including AFB and fungal cultures). I would not start HIV therapy at this point due to high risk of IRIS. I think we need to get a better handle overall on his infections before doing much else. He is on broad spectrum antimicrobial and anti fungal therapy for now, which seems reasonable. I would also probably keep the steroids where they are until we can flush out a little more diagnostically, though if he continues to clinically worsen, we may have no choice but to empirically increase them in the next 24 hours or so (which would be okay as long as he remains on broad spectrum antimicrobial therapy).
In addition to the orders already pending, I would also check EBV PCR in the blood.
From: Christian Wysocki <Christian.Wysocki at UTSouthwestern.edu<mailto:Christian.Wysocki at UTSouthwestern.edu>>
Date: Monday, August 6, 2018 at 9:05 AM
To: Dheepa Sekar <DHEEPA.SEKAR at phhs.org<mailto:DHEEPA.SEKAR at phhs.org>>, "adult-hlh-mas at lists.utsouthwestern.edu<mailto:adult-hlh-mas at lists.utsouthwestern.edu>" <adult-hlh-mas at lists.utsouthwestern.edu<mailto:adult-hlh-mas at lists.utsouthwestern.edu>>
Cc: Rosechelle Ruggiero <Rosechelle.Ruggiero at UTSouthwestern.edu<mailto:Rosechelle.Ruggiero at UTSouthwestern.edu>>, David Grinsfelder <DAVID.GRINSFELDER at phhs.org<mailto:DAVID.GRINSFELDER at phhs.org>>
Subject: Re: [Adult-hlh-mas] Possible HLH Case Parkland [securemail]
If this is histo driving this inflammatory syndrome, it should improve relatively quickly with treatment of the histo.
My concern is that although his fever curve is improving a bit over the past couple of days, he still sounds very unstable.
Hard to know what his baseline CD4 count really is, as he's actively critically ill, but if it's really this low, he could have more than just Histo.
There are enlarged lymph nodes which look accessible in the axilliae. If not turning around with amphotericin and the other broad spectrum antimicrobials, I think a LN biopsy (excisional) is warranted to rule out lymphoma/lymphoproliferative d/o.
He's on stress dose hydrocortisone, which could be increased to dexamethasone 10mg bid until he stabilizes, although I would suggest getting the LN excised before cranking up his steroids, if can be done within the next 24 hrs.
That's my opinion. Others?
From: Dheepa Sekar
Sent: Sunday, August 5, 2018 3:07 PM
To: adult-hlh-mas at lists.utsouthwestern.edu<mailto:adult-hlh-mas at lists.utsouthwestern.edu>
Cc: Rosechelle Ruggiero; David Grinsfelder
Subject: [Adult-hlh-mas] Possible HLH Case Parkland [securemail]
Alpha, U (no name given on admission and patient somewhat disoriented. Family now here and involved in care)
36 yo Honduran man presented with 1 month of fatigue and night sweats. No prior known medical history, found to have HIV and disseminated histo on this admission. Course has rapidly progressed, now intubated with ARDS and in septic shock on escalating pressor requirement. Currently on amphotericin for histo as well as RIPE (pending AFB smears) and Bactrim IV for possible PCP. Also with +RPR and treating for secondary syphilis (macules on palms).
Workup thus far:
thrombocytopenia (20, no other signs of hemolysis), anemia
HIV +, CD4 13. histo ag positive. fungal comp fix pending. fungal culture pending.
MTB CPR neg. AFB 2/3 neg
PGY3 Internal Medicine
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