[Adult-hlh-mas] [External] Re: Securemail Clements

FATEMEH EZZATI FATEMEH.EZZATI at UTSouthwestern.edu
Sat Aug 25 21:44:37 CDT 2018


Thanks Weina for the update.
I started the service today.
He felt much better with steroid with significant improvement of inflammatory markers and Anakinra was started yesterday. He was feeling great today.
Please update us with the final path report.

Negin

Sent from my iPhone

On Aug 25, 2018, at 6:57 PM, Weina Chen <Weina.Chen at UTSouthwestern.edu<mailto:Weina.Chen at UTSouthwestern.edu>> wrote:


Hi all,


An update on the bone marrow biopsy:

- Morphologically there is no overt evidence of hemophagocytosis, nor obvious lymphoma.


- However, concurrent flow cytometric analysis on BM aspirate revels:

  "A large subset of variably sized T-cells (with a high proportion of medium-sized cells) that are CD4(partial dim +)/CD8(+) and that show mild immunophenotypic variation.  These account for 22% of total events."
The nature of this population is uncertain: an expanded subpopulation of reactive T-cells?  a T-lineage lymphoproliferative disorder?"

- We are adding a couple of more markers by flow and IHC including EBV, and will inform the team with final interpretation hopefully on next Monday or Tuesday.

Thank you,
Weina







________________________________
From: Brittany Ahmed
Sent: Friday, August 24, 2018 11:18 AM
To: Bonnie Prokesch; Christian Wysocki
Cc: Adult-hlh-mas at lists.utsouthwestern.edu<mailto:Adult-hlh-mas at lists.utsouthwestern.edu>; Wende Wells; Ellen Araj
Subject: Re: [Adult-hlh-mas] [External] Re: Securemail Clements


Thank you all. I have ordered the cytokine panel, IL-18 level, EMV PCR, CMV PCR.


I agree that I bet he had some sort of inciting infection that probably triggered all this... which we will probably never know. :(


He completed day 3 of pulse dose steroids today and his inflammatory markers are much improved across the board (ferritin is down to 25000 today!), so I think we will likely start Anakinra.


Dr. Ezzati is actually on Rheum consults starting tomorrow so we will have a direct line to the team!


Thanks for all your help.


-Brittany

________________________________
From: Bonnie Prokesch <Bonnie.Prokesch at UTSouthwestern.edu<mailto:Bonnie.Prokesch at UTSouthwestern.edu>>
Sent: Thursday, August 23, 2018 7:34:41 PM
To: Christian Wysocki
Cc: Brittany Ahmed; Adult-hlh-mas at lists.utsouthwestern.edu<mailto:Adult-hlh-mas at lists.utsouthwestern.edu>; Wende Wells; Ellen Araj
Subject: [External] Re: [Adult-hlh-mas] Securemail Clements

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Hi Brittany,
This is a complicated case. Thanks so much for posting! I see that Dr. Luby has been seeing the patient (whom he knows from clinic), and I discussed the case with Dr. King (the ID attending who has been following as well). The whole story does seem most consistent with adult stills. I personally think that the positive brucella IgM was a false positive most likely and probably a red herring. The only part that doesn't quite add up to me is his leg abscess (which reportedly was not hot or swollen or tender, which is odd). Cultures were negative, but he had received some antibiotics prior to drainage on 8/12/18. The infectious work up seems to be pretty comprehensive, and my gut feeling is that while there may have been an infectious trigger acutely, in general infection is not the main issue. I agree that for completeness (and especially in the setting of steroids) checking CMV and EBV PCR is a good idea. Per Dr. King, the patient dramatically improved with steroids overall today clinically, which is good. I agree with Chris that anakinra might be the best next step (and in light of the unrevealing infectious work up, I do not think there is an ID contraindication giving it at this point).

Best,
Bonnie

On Aug 23, 2018, at 6:10 PM, Christian Wysocki <Christian.Wysocki at UTSouthwestern.edu<mailto:Christian.Wysocki at UTSouthwestern.edu>> wrote:

Hi Brittany,
Thank you for posting this case. Another potential Stills!!......
Hematolymphoid malignancy should be aggressively ruled out, and looks like BMBx was done today, and imaging is negative for LAD or splenomegaly.
A big infectious w/u has been done and looks negative although I'd definitely add EBV PCR as well as CMV PCR, and would love to hear Bonnie's opinion if she's available.
I would suggest an IL18 level as an indirect marker of inflammasome hyperactivation (would support use of anakinra).  Would also advocate for a cytokine panel (does not include IL18 unfortunately, but does include IL6, which might argue more in favor of Actemra).
Wouldn't necessarily wait to get the IL18 result back to start anakinra, but in case things respond weirdly (as we have seen recently w Ms. Lowder), at least we'd have a full set of baseline data to guide us.  I have included Dr. Araj and Wende Wells, to make them aware that these labs may be coming.

-Chris

From: Brittany Ahmed
Sent: Thursday, August 23, 2018 2:01 PM
To: Adult-hlh-mas at lists.utsouthwestern.edu<mailto:Adult-hlh-mas at lists.utsouthwestern.edu>
Subject: [Adult-hlh-mas] Securemail Clements


Hello,

I have a patient at Clements on whom we wanted to get the task force's opinion.

MRN 91897375

Ferrer, Jose

Jose Ferrer is a 44 year old Filipino male physician with hyperlipidemia, hypertension, and recent admit (8/10/2018 - 8/17/2018) for fever of unknown origin suspected to be due to an infectious cause. The fevers had been ongoing for 5 weeks prior to that admission, along with chills, sweats, generalized weakness, arthralgias, and myalgias. He has had extensive travel to India, Kenya, Tanzania, Senegal, and the Philippines. He was asymptomatic while abroad, but the symptoms started on arrival to the US.

Briefly, during recent admit, he had extensive evaluation was found to + Brucella Ab IgM along with left knee arthritis (aspirated, no growth), right shoulder arthritis (aspirated, no growth), and left calf abscess that underwent I&D by general surgery. He was discharged on ceftriaxone + rifampin + doxycycline.


He was readmitted with symptoms of fever (104.6 max), loose stools, and nausea that had not improved since discharge. Antibiotics were held as it was thought that this could be adult onset Still's Disease.


Labs on admission:
ESR 100
CRP 142.6
Ferritin 45621
Fibrinogen 509
LDH 720


Heme/Onc was consulted and they will perform BM biopsy. Soluble IL-2 receptor is pending in lab.


So far, we have started him on Solu-Medrol 1000 mg on 8/22.


Thank you for review of this case!

Brittany Ahmed
Rheumatology Fellow PGY V

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