[PAGID] Eosinophilic abscesses in a CGD patient. .

Sergio Rosenzweig srosenzweig at garrahan.gov.ar
Tue Jun 8 08:50:39 EDT 2010


If positive, Quantiferon Gold (QG) test would add mycobacterial
specificity to the previous strongly positive PPD test: it detects MTB
complex (except M. bovis BCG) and not NTMs. Besdies, the read out (IFNg)
is different than PPD (DTH). I never tried QG on CGD or MSMD patients,
my impression is it might work fine on CGD, and also on IFNGR1 and 2
deficiencies (high IFNg producers) but probably not on IL12/23 signaling
pathway defects.
Sergio


Sergio D. Rosenzweig, MD, PhD
Chief, Infectious Diseases Susceptibility Unit
Laboratory of Host Defenses, NIAID, NIH
10 Center Dr., Bldg. 10, CRC 5W-3888
Bethesda, MD 20892-1456
Phone (301) 451 8971
Fax (301) 451 7901
Cell (240) 361 7617
Pager 102 10678
srosenzweig at niaid.nih.gov

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>>> Sabiha Anis <sabiha_anis at hotmail.com> 06/08/10 2:54 AM >>>


I agree with Dr Ashish on the reliability of Quantiferon Gold test in
this case. If the patient is giving a strong positive result with PPD
then this test will not resolve the issue if there is already a
hyerreactive immune system.



Regards

Sabiha Anis
Clinical Immunologist

Sindh Institute of Urology and Transplantation

Karachi, Pakistan


> Date: Wed, 2 Jun 2010 13:48:54 -0400

> From: Ashish.Kumar at cchmc.org

> To: pagid at list.clinimmsoc.org

> Subject: Re: [PAGID] Eosinophilic abscesses in a CGD patient. .

>

> Is Quantiferon useful/reliable in patients with CGD or 1L-12/IFN-g

> pathway defects? Specifically, is the negative predictive value good

> enough?

> Ashish

>

> Ashish Kumar, MD, PhD

> Assistant Professor

> Cincinnati Children's Hospital Medical Center

> Cincinnati, OH

>

>

>

> >>> Howard Lederman <hlederm1 at jhmi.edu> 6/2/2010 1:36 PM >>>

> Mary Ellen,

>

> Quantiferon gold is a commercial in vitro test. Whole blood is mixed

> with TB Ags. Read-out is interferon-gamma production. Here's a link to

> a page at the CDC website.

> http://www.cdc.gov/tb/publications/factsheets/testing/QFT.htm

>

> Howard

> Howard M. Lederman, M.D., Ph.D.

> Professor of Pediatrics, Medicine and Pathology

> Division of Pediatric Allergy and Immunology

> Johns Hopkins Hospital - CMSC 1102

> 600 N. Wolfe Street

> Baltimore, MD 21287-3923

> Phone: 410-955-5883

> Fax: 410-955-0229

> Email: Hlederm1 at jhmi.edu

>

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>

> -----Original Message-----

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

> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Conley, Mary

> Ellen

> Sent: Wednesday, June 02, 2010 1:15 PM

> To: 'pagid at list.clinimmsoc.org'

> Subject: Re: [PAGID] Eosinophilic abscesses in a CGD patient. .

>

> Hi Steve,

> Can you explain to some of us ignorant (but interested) folks what a

> quantiferon gold is?

> Mary Ellen

>

>

> Mary Ellen Conley, MD

> Department of Immunology/ Mail Stop 351

> St. Jude Children's Research Hospital

> 262 Danny Thomas Place

> Memphis, TN 38105-3678

> FAX 901-595-3977

> TEL 901-595-2576

>

>

> -----Original Message-----

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

> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Holland,

> Steven (NIH/NIAID) [E]

> Sent: Wednesday, June 02, 2010 11:41 AM

> To: Howard Lederman; pagid@> Was the material cultured for Granulibacter and similar organisms? I

> doubt Churg Straus but stopping mesalamine in the short run seems

> harmless enough. Necrotizing granulomatous abscesses in CGD should be

> due to a bacterial or fungal infection. I would treat him empirically

> with ceftriaxone and vori, adding meropenem if he were acutely ill. If

> he is from Maryland then M. marinum or another NTM could do this,

> although 15 mm is pretty big. I would send a quantiferon gold.

>

> Steve

>

>

> On 6/2/10 12:29 PM, "Howard Lederman" <hlederm1 at jhmi.edu> wrote:

>

> Colleagues,

>

> I have a 4 ½ year old male patient who was diagnosed with chronic

> granulomatous disease at 5 weeks of age after presentation with

> bilateral neck masses (cervical lymphadenitis caused by

> methicillin-sensitive S. aureus). He has had multiple episodes of

> cervical lymphadenitis, requiring parenteral antibiotics. He is

> maintained on interferon gamma (3x/week) and Ciprofloxacin (TMP/SMZ

> caused leukopenia and thrombocytopenia). Other medications are iron

and

> multivitamin daily.

>

> He has a history of bloody stools in early infancy thought to be due

to

> cow's milk enterocolitis. Symptoms resolved when he was switched to an

> elemental formula, and a colonoscopy while on Elecare was normal. He

> has introduced to cow's milk at 12 months of age without problems.

>

> When he was 3 ½ yrs old, he had bloody stools and poor weight gain.

> Cultures, and stool test for ova and parasites were normal. RASTs to

> food allergens were negative. Milk elimination did not help. In Jan

> 2010, EGD showed chronic duodenitis, and colonoscopy showed many areas

> with crypt distortion, prominent lamina propria eosinophils and rare

> intraepithelial eosinophils. Granulomas were not seen. He was started

> on Apriso (mesalamine) in early April to treat inflammatory bowel

> disease.

>

> At the end of April, he developed fevers to 103 F with no obvious

> source. Over a two week period, his WBC increased to WBC 30,000 with

> 44% polys, 43% lymphs and 6% eos. CRP 16 mg/dL. He then developed

> abdominal distension and a new hydrocele over a short period of time,

> due to bulky lymphadenopathy involving the mesentery and

> retroperitoneum. A PPD was placed and was positive with 15 mm

> induration.

>

> He underwent an excisional biopsy of several mesenteric nodes so that

> we could identify the mycobacterium that we were confident was the

cause

> of his acute symptoms. No acid fast organisms were seen in the lymph

> nodes. Cultures are still negative but it has been only 2 weeks so

> far. However, the lymph node histology was a complete surprise to us.

> There were granulomas, but the center of many of them were filled with

> abscesses of eosinophils! [The CIS has posted a photomicrograph of

> the lymph node biopsy at

> http://www.clinimmsoc.org/UserFiles/file/pagid_lederman.pdf]

>

> Our pathologists favor a diagnosis of Churg-Strauss based only upon

the

> histology. The Infectious Diseases group thinks that this represents

> a drug hypersensitivity reaction and wants us to stop Apriso. Neither

> diagnosis explains the positive PPD.

>

> Does anyone have a differential diagnosis for this?

>

> Howard

> Howard M. Lederman, M.D., Ph.D.

> Professor of Pediatrics, Medicine and Pathology Division of Pediatric

> Allergy and Immunology Johns Hopkins Hospital - CMSC 1102 600 N. Wolfe

> Street Baltimore, MD 21287-3923

> Phone: 410-955-5883

> Fax: 410-955-0229

> Email: Hlederm1 at jhmi.edu <mailto:Hlederm1 at jhem.jhmi.edu>

>

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>

> Chief, Laboratory of Clinical Infectious Diseases CRC B3-4141 MSC 1684

> Bethesda, MD 20892-1> Assistant: Adrienne Woodworth

> 301-451-9019 (v)

> 301-480-4506 (fax)

> awoodworth at niaid.nih.gov

>

>

>

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