[CIS-PAGID] FW: XLA / helicobacter

[Charlotte Cunningham-Rundles@mssm.edu]

Was this one published?


On 7/25/11 6:12 PM, "Dowling, Paul MD" <pdowling at cmh.edu> wrote:


> Dear Charlotte,

> 

> I also had a case like this. This patient had a diagnosis of XLA and was doing

> well until age 16 when he started having unexplained painful "bumps" on his

> arms and legs. They were small at first, warm, painful and would last a few

> days on his arms and lower legs.  No fever, change in CBC etc. was seen. they

> initially would clear completely after a few days. He thought they occured

> from trauma as he played soccer and they occurred in areas usually hit by the

> ball. Later as they became more frequent they were thought to be some type of

> vasculitis or atypical cellulitis. Attempts at culture and biopsy showed no

> organisms. He was treated with a large variety of antibiotics by ID without

> improvement. Bx was consistent with panniculitis.  A couple lesions on the

> lower legs persisted and started necrosing...leaving large non healing ulcers.

> After speaking with a couple experts on XLA the thought of

> Heliobacter/Flexispira was entertained although all attempts at culturing were

> futile.   The pateint was eventually sent to the NIH where cutures were also

> negative but the flexispira organisms were seen by staining the biospies with

> "Warthin-Starry ?" Stain. He was treated with a PICC line with IV Tobramycin

> and Meropenem for many months. His lesions started healing. During treatment

> Tobra was dc'ed as he developed tinnitus and documented hearing loss and was

> switched to po doxycycline along with the IV Meropenem. He was treated about

> 10 months until he stopped meds himself and remained well for approximately a

> year until lost to followup.

> 

> Hope this adds a little to your data base on these patients.

> 

> Paul

> 

> Paul J Dowling, MD

> Training Program Director, Allergy/Immunology

> Associate Professor, Dept.of Pediatrics

> University of Missouri - Kansas City

> Children's Mercy Hospitals and Clinics

> 816-234-3097 (office); email pdowling at cmh.edu

> 

> 

> ________________________________

> From: Ciaccio, Christina, E

> Sent: Saturday, July 23, 2011 5:09 PM

> To: Dowling, Paul MD

> Subject: Fwd: [CIS-PAGID] XLA / helicobacter

> 

> 

> Did you have a case like this?

> 

> Begin forwarded message:

> 

> From: "Charlotte 

> Cunningham-Rundles at mssm.edu<mailto:Cunningham-Rundles at mssm.edu>"

> <charlotte.cunningham-rundles at mssm.edu<mailto:charlotte.cunningham-rundles at mss

> m.edu>>

> Date: July 23, 2011 9:38:23 AM CDT

> To: PAGID <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>

> Subject: [CIS-PAGID] XLA /  helicobacter

> Reply-To: "pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>"

> <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>

> 

> Hi,

> 

> We have a case of a 25 year old man with leg swelling, pyoderma gangrenosum,

> gram neg bacteremia but no fever or increased WBC,   that I think it will

> prove to be a Helicobacter. He is not yet proven to have XLA but has no B

> cells and I assume with profound hypogammaglobulinemia and early onset, this

> is likely what he has.

> 

>   I know there are cases reported around the US and world, but wonder, if we

> as a group:

> 

> 

>  1.  Can make a rough count of the cases?

>  2.  Say what antibiotics worked in the long run?

>  3.  Are there any cases in CVID or other congenital immune defects?

> 

> Charlotte

> 

> Charlotte Cunningham-Rundles MD PhD

> Department of Medicine

> Immunology Institute

> Mount Sinai School of Medicine

> 1425 Madison Avenue

> New York City, New York 10029

> 

> 212 987 5593 fax

> 212 659 9268 telephone

> 

> 

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