[CIS PIDD] [cis-pidd] Fw: HLH in SCid with CMV?

Thu Nov 2 15:52:11 EDT 2017

I was just wondering if there could be a role for anakinra in this situation, esp if you were avoiding steroids.

Mark Gorelik, MD

Children's Hosp of San Antonio

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Sent: Thursday, November 2, 2017 2:12:11 PM
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Subject: Re: [cis-pidd] Fw: HLH in SCid with CMV?

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Thank you so much for the advice. I gave 1 gram/kg of the IVIG (5 grams) - rather than 0.5 g/kg, which seemed to result in significant improvement in his CBC. I will consider giving a second dose as well. I briefly had him on 2 g/kg/day of prednisone (more so as an addition for empiric PJP treatment ), but was worried in the context of his significant CMV viremia (once those results returned), so stopped it. We are awaiting the HLA typing results and trying to control the infection, but will certainly not delay definitive treatment while awaiting the genetic panel.

Thank you again,

Tamar

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From: cis-pidd at lyris.dundee.net <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Thursday, November 2, 2017 12:59:43 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Fw: HLH in SCid with CMV?

Dear Tamar,

Partial or full-blown presentations of (2') HLH can occur in (S)CID patients, typically infection associated.

In addition to CMV directed therapy high dose (2g) IVIG may help you to dampen the immune activation. I would also not hesitate to use steroids. If things threaten to get out of control, conditioning for haplo rather than etoposide would be my last resort.

Do not wait for genetics for your clinical decisions!

Best wishes, Stephan

Von meinem iPhone gesendet

Am 02.11.2017 um 17:35 schrieb CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>:

Hello all - my apologies if this is being sent a second time, but I am not sure if the first email went through.

I would greatly appreciate your expert management advice on a 3 month old boy with suspected SCID and disseminated CMV infection, with some HLH-like features.

My main question is regarding treatment for immune activation versus simply trying to treat infection (and of course any other suggestions for managing the severe infection). On a secondary note- what is the role of holding breastfeeding  in the context of established CMV infection?

Here is the story:

3 month old boy with some HLH features but not meeting criteria: Ferritin >3500, pancytopenia with hemoglobin 70 g/L, platelets 20 x 10^9/L, WBC 2-5 x 10^9/L, hypofibrinogenemia, hypertriglyceridemia, massive hepatomegaly, normocellular bone marrow in keeping with inflammatory/reactive process without hemophagocytosis; no fever and no splenomegaly at any point, normal soluble IL2R levels). Not enough T cells or NK for Cd107a, Perforin/granzyme expression. He has prominent neurologic symptoms (seizures, irritability) with increased protein and cell count in CSF (evenly lymphocytes, neutrophils and monos/macro), but no infections identified there (CMV PCR pending). He has rising liver enzymes (AST, GGT both in hundreds). MRI/CT brain normal. Worsening bilateral infiltrates and ground glass opacities on CXR and CT chest, small effusion. He is on low flow oxygen.

His immune workup showed almost no T or NK cells (36 cells/mcl T cells) on two occasions, but normal B cells in peripheral blood (similar distribution in the marrow). He has low IgG 1.5 g/L, absent IgA, and normal IgM (0.27 g/L). No thymus seen on CT/CXR (acknowledging he is sick/stressed), no secondary lymphoid tissue on exam. Diarrhea since receiving rotavirus 1 month ago (however no rotavirus in stool- repeat pending).

Pending investigations: SAP, XIAP, CD132 by flow; mitogen proliferations, TRECs (retrospectively since we don’t screen in this province yet), CD45Ra/Ro/recent thymic emigrants, bronchoscopy infectious results, Genetic panel for SCID.

Current management includes:

-IVIG replacement recently given

-Septra PJP treatment dose

-Gancyclovir

-Ceftriaxone

Thank you so much!

Sincerely,

Tamar Rubin

Tamar Rubin, MD, FRCPC

Pediatric Clinical Immunology and Allergy

University of Manitoba

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