[CIS PIDD] CGD patient

[Richard Wasserman]

2g/kg/d for four days is a very high dose. Hyperviscosity, which is
virtually never a problem in PID patients, is a distinct possibility and
could certainly have created a risk for stroke. The value of IGIV in
hemodynamically unstable bacteremia and sepsis is, at best, uncertain.
Charlotte's point about RE blockade is relevant as well.
Richard Wasserman
Dallas

On Sat, Jun 16, 2012 at 12:31 PM, Santhosh Kumar/FS/VCU <skumar at vcu.edu>wrote:


> His fibrinogen and ddimer were normal in the beginning and is just

> starting to go up.

>

>

> Santhosh Kumar, MD

> Assistant Professor,

> Allergy & Immunology

> Ph-  804-628-1605

> Fax-804-828-1751

>

>

> -----pagid-bounces at list.clinimmsoc.org wrote: -----

>

> To: "<pagid at list.clinimmsoc.org>" <pagid at list.clinimmsoc.org>

> From: "Kleiner, Gary" **

> Sent by: pagid-bounces at list.clinimmsoc.org

> Date: 06/16/2012 10:51AM

>

> Subject: Re: [CIS PIDD] CGD patient

>

> We have seen sepsis DIC and necrosis of hands and feet in this setting of

> cgd

>

>  Was the child's fibrinogen and d dimers normal?

>

>

>

> Gary Kleiner MDPhD

>

>

> On Jun 16, 2012, at 10:00 AM, "Santhosh Kumar/FS/VCU" <skumar at vcu.edu>

> wrote:

>

>   Hello everyone,

>

> ** **

>

> I have a newly diagnosed CGD patient whom I am seeing as a consult.

>

> He is a 2y/o boy, first child, who was recently diagnosed with CGD (most

> likely x-linked), DHR confirmed, mom is a carrier. Gene studies are

> pending. He developed B.Cepacia sepsis, respiratory failure, SIRS. He grew

> B.cepacia in blood and ascitic fluid. He is currently intubated and

> responding well to antibiotics. Yesterday he had seizures and CT-scan and

> MRI showed b/l extensive cerebral infarct with sparing of midbrain. MRA and

> MRV were normal. We think the stroke he had was probably related to

> hypotension, although there was no prolonged hypotensive period documented.

>

> ** **

>

> His labs so far:

>

> WBC-8400, hb-9.4/27.9

>

> PT-13.6

>

> INR-1.3

>

> apTT-31s

>

> Initial IgG- 789—now- 1194 (post IVIG infusions)

>

> Ferritin- 18409- now-1076

>

> AST- 2259- now-144

>

> ALT-289-now-44

>

> Creatinine-0.85

>

> IL-2 receptor alpha-23,890

>

> Bone marrow analysis prior to him developing respiratory failure was

> normal without any hemophagocytes.

>

> ** **

>

> At the onset of respiratory failure and sepsis, he had high levels of

> ferritin, IL-2rec alpha levels, and there was some concern for HLH. He was

> started on solumderol 4mg/kg and IVIG 2gm/kg daily (although not

> particularly for HLH). His inflammatory parameters including ferritin

> responded well to this regimen and antibiotics.

>

> ** **

>

> My questions:

>

> 1.     Was his initial picture consistent with HLH. Should I have done

> anything different in terms of his treatment

>

> 2.     Any thoughts on CGD and HLH association

>

> 3.     anyone have any comments on the massive stroke this child

> developed.

>

> Appreciate any input.** **

>  Santhosh Kumar, MD

> Assistant Professor,

> Allergy & Immunology

> Ph-  804-628-1605

> Fax-804-828-1751

>

>   **

>

>



-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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