[PAGID] CVID/HIGM/ALPS

[Brian Vickery]

Interesting case, Lisa. I don't know much about Achromobacter but in reading
about it I came across a case report of a CD40-deficient patient with
lymphadenopathy as the source of persistent Achromobacter bacteremia. I'd
keep looking for other causes of HIGM:

Clinical Infectious Diseases 2000;31:1183–1187

DOI: 10.1086/317461
Recurrent *Achromobacter xylosoxidans* Bacteremia Associated with Persistent
Lymph Node Infection in a Patient with Hyper–Immunoglobulin M Syndrome

Jörn-Hendrik Weitkamp,1 Yi-Wei Tang,2,3 David W. Haas,2, 4Narinder K. Midha,
3 and James E. Crowe, Jr.1,4

Departments of 1Pediatrics, 2Medicine, 3Pathology, and 4Microbiology and
Immunology, Vanderbilt University Medical Center, Nashville, Tennessee

*Achromobacter xylosoxidans* (formerly *Alcaligenes xylosoxidans*) is a rare
but important cause of bacteremia in immunocompromised patients, and strains
are usually multiply resistant to antimicrobial therapy. We report an
immunocompromised patient with hyper–immunoglobulin M syndrome who suffered
from 14 documented episodes of *A. xylosoxidans* bacteremia. Each episode
was treated and resulted in rapid clinical improvement, with blood cultures
testing negative for bacteria. Between episodes, *A. xylosoxidans* was
isolated from an excised right axillary lymph node, whereas the culture of
the central venous catheter, removed at the same time, was negative.
Multiple cultures from sputum, stool, and urine samples, as well as from
gastrointestinal biopsies or environmental sources, were negative. Results
from antibiotic sensitivity testing and pulsed-field gel electrophoresis
suggested that a single strain of *A. xylosoxidans* caused the recurrent
bacteremias in this patient; this strain originated from persistently
infected lymph nodes. Lymphoid hyperplasia is a prominent characteristic of
hyper-IgM syndrome and may serve as a source of bacteremia with
low-pathogenicity organisms.
Best regards,
Brian

Brian Vickery, MD
Instructor
Division of Pediatric Allergy & Immunology
Duke University School of Medicine
919.684.9083 (ofc)
919.970.9095 (pgr)



On Thu, Aug 7, 2008 at 10:45 AM, Lisa Kobrynski, MD, MPH
<lkobryn at emory.edu>wrote:


> I was hoping to get some suggestions on a patient of mine

> I am following a 10 year old boy who presented initially with lymphadenitis

> and

> bronchiectasis at age 4 years.  He had malakoplakia found by the

> pathologists in

> his tonsillar tissue after a tonsillectomy.

>

> He initially had low IgG and IgA with a normal IgM and very poor specific

> antibody responses and was given a diagnosis of CVID.

> Over the years he has had assorted infections (yersinia, salmonella

> enteritis,

> pneumonia) but developed very significant cervical lymphadenopathy last

> year.

> Repeated biopsies have not shown any malignant transformation or clonality

> of

> the cells.  One node became necrotic and grew achromobacter xylosidans,

> which he

> has had intermittently in the blood ever since.  It is not resistant to

> everything.  Also his IgM is now over 3000.  ALPS panel initially showed an

> increase in DN T cells, activated T cells and a decrease in memory B cells.

>  DN

> T cell % had decreased at the last measurement.

> He has minimal hilar adenopathy, some mild inguinal adenopathy, but the

> cervical

> LN are the biggest issue.

>

> I want to take a poll and find out who thinks this is just CVID with

> lymphoproliferation?  SHould we treat him with chemotherapeutic agents to

> suppress the lymphoproliferation?

> Could this have been ALPS all along?

> WHo thinks this is a HyperIgM - AID or UNG defect?  (We checked CD40 and

> CD40L

> and they were normal)

>

> Thanks

> Lisa

>

> Lisa Kobrynski, MD, MPH

> Associate Professor of Pediatrics

> Division of Allergy and Immunology

> Emory University

> 404-727-3575

> 404-727-5045 (fax)

>

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