[CIS PIDD] CGD patient

[Church, Joseph]

At presentation did he have other indicators of HLH?
            - leukopenia
            - thrombocytopenia
            - spiking fevers
            - organomegaly

Re HLH in CGD:  see Parekh C et al.  Hemophagocytic Lymphohistiocytosis in Children with Chronic Granulomatous Disease.  Pediatr Blood Cancer 2011; 56:460-2.

Joe Church
Children's Hospital Los Angeles

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Cunningham-Rundles, Charlotte
Sent: Saturday, June 16, 2012 8:00 AM
To: PAGID
Subject: Re: [CIS PIDD] CGD patient

Or that RES blockade due to IVIG was not helpful in overwhelming infection.


On 6/16/12 10:39 AM, "drrichwasserman at gmail.com" <drrichwasserman at gmail.com> wrote:
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 <tel:804-628-1605>
Fax-804-828-1751 <tel:804-828-1751>



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