[PAGID] FW: ? Granulomatous Angiitis

[Vogler, Larry B]

Thanks very much for your all of your comments so far.
I do not currently have the answers to all of the questions posed, but will try to obtain them.
We will definitely look into a defect in V-D-J recombination. I don't believe that she had an SPEP run and her biopsy tissue was not stained for immunoglobulins, CHOP did not look at TLRs.  Our pathologist states that the predominant cell type in the lesions is histiocytes, with some neutrophils and relatively few eosinophils and lymphocytes.  The patient has a normal head size and neurological development. I will keep you posted on the results of further investigations.
Larry Vogler

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of pagid at list.clinimmsoc.org
Sent: Wednesday, September 30, 2009 9:41 AM
To: Vogler, Larry B; pagid at list.clinimmsoc.org
Subject: Re: [PAGID] FW: ? Granulomatous Angiitis
Importance: Low

A couple of questions on the IgG- I assume there is no monoclonal spike ?  What is her EBV status ?

Then: Was the lesion stained for Ig's ?  Have you or CHOP or someone done a TLR screen ?

Melvin Berger, M.D., Ph.D.
Adjunct Professor of Pediatrics and Pathology
Case Western Reserve University
phone 216 844 3237

Director, Jeffrey Modell Center for Primary Immune Deficiencies
Division of Allergy-Immunology
Rainbow, Babies and Children's Hospital
University Hospitals of Cleveland
RB&C Rm 504, MS 6008B
11100 Euclid Ave.
Cleveland, OH 44106

________________________________
From: pagid-bounces at list.clinimmsoc.org on behalf of Vogler, Larry B
Sent: Tue 9/29/2009 5:53 PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] FW: ? Granulomatous Angiitis
[cid:~WRD000.jpg]

I would very much appreciate any help that you may provide in the diagnosis and management of this patient.

The patient is a 10 year old East Indian girl with a normal birth.  She had some episodes of pharyngitis and gastroenteritis after age 4 months and was hospitalized at age 10 months at Children's Healthcare of Atlanta-Egleston with well-confirmed adenoviral hepatitis. At 19 months in 12/00 she had an episode of IgG auto-immune hemolytic anemia. In 12/03 she had pneumonia and in 3/05 bronchitis and otitis media. She had an ear drum perforation in 5/06 and was diagnosed with asthma in 4/08. In 11/08 she was noted to have growth retardation and nail clubbing.  A chest CT showed extensive bronchiectasis. In 1/09 she developed swelling of her left elbow and left ankle associated with tender subcutaneous nodules. An MRI showed lytic lesions in the distal humerus and increased marrow signal in the proximal left radius.
She has had high ESRs (118) and the following additional labs: WBC 4.0: 69% segs, 17% lymphs, 7% monos, 7% eos, Hgb 9.3, platelets 234. IgG 2280, IgA 60, IgM 92, IgE <2, CH50 51. CD4 lymphocytes 27% (absolute ct.  295), CD8 9% (101), CD 19 14% (164), CD 16/56 37% (420). Good responses to PHA and Con A in vitro, normal NK killing in vitro and normal neutrophil oxidative burst.  ANCA was negative, ANA + 1:160, ACE 20 (nl).  TB skin test negative, and negative routine, fungal and AFB cultures and serologies for Brucella, Bartonella, Histoplasma and HIV.
She has been evaluated at MCG, CHOP and Emory. A biopsy of an elbow soft tissue lesion on 5/11/09 was interpreted by our pathologist as showing necrotizing angiocentric granulomatous inflammation with granulomatous angiitis of small to medium-sized arterioles. A recent bronchoscopy  showed signs of chronic bronchial inflammation but no organisms. An abdominal ultrasound showed modest splenomgaly with normal echogenicity and a brain MRI was normal.  A recent ophthalmology exam revealed no uveitis or retinal lesions.  Attached are photos of the skin lesions and histopathology.
We believe that she has necrotizing sarcoid granulomatosis as described in the following Pub Med articles: PMID 17377369, 12823717, 12784179, 12687600, 10232451, 8092646.  However, this is not certain and we are reluctant to immunosuppress this young girl with low lymphocyte numbers if other diagnoses are likely.

Thanks for your thoughts.
Larry


Larry B. Vogler, M.D.
Director, Division of Rheumatology and Immunology
Department of Pediatrics
Emory University
2015 Uppergate Drive
Atlanta, GA 30322
phone: 404-778-2400
fax: 404-727-3757



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