[CIS PIDD] CGD patient

[Richard Wasserman]

What was the rationale for the immunomodulatory dose of IgG. Was it given
as a single dose? How quickly was it given. One must consider the
possibility that the stroke was an IgG side effect.
Richard Wasserman
Dallas

On Sat, Jun 16, 2012 at 9:00 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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