[PAGID] Eosinophilic abscesses in a CGD patient. .

[Howard Lederman]

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 at list.clinimmsoc.org
Subject: Re: [PAGID] Eosinophilic abscesses in a CGD patient. .

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 20892-1684
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