[CIS PIDD] [cis-pidd] Follow up for post -HCT XIAP patient

Wed Aug 23 11:45:14 EDT 2017

I am happy to see him at Wake.

Jason W Caldwell DO FAAAAI
Associate Professor of Internal Medicine and Pediatrics
Section of Pulmonary, Critical Care, Allergic and Immunological Diseases
Program Director of Allergy/Immunology Fellowship
Wake Forest University School of Medicine
Office: 336-716-5166
Administrative: 336-716-4843
Pager: 336-806-8330
jcaldwel at wakehealth.edu<mailto:jcaldwel at wfubmc.edu>
[Wake Forest School of Medicine]

From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Wednesday, August 23, 2017 10:39 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: [cis-pidd] Follow up for post -HCT XIAP patient

HI

I have a 20y/o XIAP patient who is doing well 2 years post HCT. He is moving to North Carolina in the Charlotte area this spring. Can anyone recommend an immunologist/transplant group to continue his followup for post-HCT immune function and late effects monitoring?

Thanks

Jen

Jen Heimall, MD
Allergy/Immunology Attending Physician
Medical Director Day Medicine
Assistant Professor of Clinical Pediatrics
Perelman School of Medicine at University of Pennsylvania

The Children's Hospital of Philadelphia
3401 Civic Center Blvd
Philadelphia, PA 19104
215-590-2549 (p)
215-590-6849 (f)

________________________________
From: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Sent: Monday, August 21, 2017 5:00 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] XIAP

He has not had hypogamm since I have been following over the last 8 years.

Jason W Caldwell DO FAAAAI

Associate Professor of Internal Medicine and Pediatrics

Section of Pulmonary, Critical Care, Allergic and Immunological Diseases

Program Director of Allergy/Immunology Fellowship

Wake Forest University School of Medicine

Office: 336-716-5166

Administrative: 336-716-4843

Pager: 336-806-8330

jcaldwel at wakehealth.edu<mailto:jcaldwel at wfubmc.edu>

From: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Monday, August 21, 2017 4:45 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: RE:[cis-pidd] XIAP

This email contains a suspicious URL

Jason

Is the patient hypogam?

James

James W. Verbsky M.D./Ph.D.

Associate Professor of Pediatrics and Microbiology

Medical Director, Clinical Immunology Research Laboratory

Medical Director, Clinical and Translational Research

Medical College of Wisconsin

Milwaukee, WI

From: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Monday, August 21, 2017 3:00 PM
To: CIS-PIDD
Subject: [cis-pidd] XIAP

Good day,

I have a 12 year old patient with confirmed XIAP.  He presented as an infant with fulminant HLH secondary to EBV.  He has done well since with no other complications until recently.

About 3 weeks ago his PCP called secondary to swelling of the left foot and ankle.  No pain, normal range of motion, but obviously swollen.

MRI:

1. No evidence of osteomyelitis of bone erosion identified. No subperiosteal abscess.

2. Ankle joint effusion and synovial enhancement noted, concerning for joint inflammation.

3. There is fluid surrounding the flexor digitorum longus tendon, with postcontrast enhancement of the synovium, findings concerning for tenosynovitis, unclear whether this is reactive to soft tissue inflammation and joint effusion/inflammation versus infected tenosynovitis.

4. Extensive subcutaneous and deep soft tissue edema of the lower leg, ankle, foot identified, medial side worse than lateral side concerning for soft tissue inflammatory changes/cellulitis. No focal fluid collection identified.

Then 1 weeks ago went to PCP for acute knee swelling and pain.  He came into our facility with difficulty putting weight on left leg at the knee, swelling, pain, and fever of 101.

MRI:

1. There are no findings to indicate osteomyelitis of left knee. Small joint effusion and synovial enhancement noted suggesting inflammatory change/septic arthritis. No articular erosions noted.

2. There is reactive edema surrounding the distal femoral anterior compartment muscles and in the proximal lead predominantly involving the posterior compartment, likely reactive changes. No focal fluid collection

Ortho did an I & D of his knee.  (did not want to look at the ankle since it was "chronic")

The I & D was remarkable for pus which was drained and closed.  He was started on antibiotics and sent home on 3 weeks of Linezolid.

He returned today with improvement in knee swelling, normal temp, but zero improvement in the ankle foot.

Labs:

                        Hgb      platelets            Sed rate            Ferritin              crp

Admission labs:  : 9.9,    693000,            64,                    1100

Discharge            9.8          653000                  64                           295                         55.5

Today                                                                    77                           414                         72.2

Triglcerdies and LDH are normal.

All cultures are no growth to date. (acid fast and fungal are no growth for 7 days.

There is mention of arthritis in these patient in the literature, but I did not see any details.

Has anyone had experience with this manifestation in XIAP?

Thoughts on treatment if cultures remain negative?

Any other thoughts??

Thank you in advance.

Jason

Jason W Caldwell DO FAAAAI

Associate Professor of Internal Medicine and Pediatrics

Section of Pulmonary, Critical Care, Allergic and Immunological Diseases

Program Director of Allergy/Immunology Fellowship

Wake Forest University School of Medicine

Office: 336-716-5166

Administrative: 336-716-4843

Pager: 336-806-8330

jcaldwel at wakehealth.edu<mailto:jcaldwel at wfubmc.edu>

---

You are currently subscribed to cis-pidd as: jverbsky at mcw.edu<mailto:jverbsky at mcw.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396710.777d26b6da96d84a8b0d5e2d208337f2&n=T&l=cis-pidd&o=4538038<BLOCKEDurldefense%5b.%5dproofpoint%5b.%5dcom/v2/url?u=http-3A__cts%5b.%5ddundee%5b.%5dnet_u-3Fid-3D96396710%5b.%5d777d26b6da96d84a8b0d5e2d208337f2-26n-3DT-26l-3Dcis-2Dpidd-26o-3D4538038&d=DwMFAg&c=aFamLAsxMIDYjNglYHTMV0iqFn3z4pVFYPQkjgspw4Y&r=feBlMS6C4RRx18fFG8bUCQ&m=WXNPwdAIb4NZB01goSiKnKePG4-3WGqrK68kyLfEXXA&s=w5Hb7LDuWYZNfdwNFKf5E7MsYTQC0PHG0Rb_O6ForyM&e=BLOCKED>

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-4538038-96396710.777d26b6da96d84a8b0d5e2d208337f2 at lyris.dundee.net<mailto:leave-4538038-96396710.777d26b6da96d84a8b0d5e2d208337f2 at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: jcaldwel at wakehealth.edu<mailto:jcaldwel at wakehealth.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=103655515.9f37f13f3aa2677fd906318bfdf81ad7&n=T&l=cis-pidd&o=4538094

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-4538094-103655515.9f37f13f3aa2677fd906318bfdf81ad7 at lyris.dundee.net<mailto:leave-4538094-103655515.9f37f13f3aa2677fd906318bfdf81ad7 at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: heimallj at email.chop.edu<mailto:heimallj at email.chop.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396616.cc2f3c8717a4b8dcca99e88dbb0e03cd&n=T&l=cis-pidd&o=4538120

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-4538120-96396616.cc2f3c8717a4b8dcca99e88dbb0e03cd at lyris.dundee.net<mailto:leave-4538120-96396616.cc2f3c8717a4b8dcca99e88dbb0e03cd at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: jcaldwel at wakehealth.edu<mailto:jcaldwel at wakehealth.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=103655515.9f37f13f3aa2677fd906318bfdf81ad7&n=T&l=cis-pidd&o=4540947

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-4540947-103655515.9f37f13f3aa2677fd906318bfdf81ad7 at lyris.dundee.net<mailto:leave-4540947-103655515.9f37f13f3aa2677fd906318bfdf81ad7 at lyris.dundee.net>

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=4541028
or send a blank email to leave-4541028-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20170823/f7136fcd/attachment-0001.html>