[CIS-PAGID] FW: XLA / helicobacter

[Cunningham-Rundles, Charlotte]

Hi Mary Ellen,

Thank you so much! 

Our pt has yet to have the final gram neg bug named, but it is out for
molecular typing now. We have him on Ertapenem for once a day dose by PICC
line. His leg is healing some. I guess we should add something more?

Is this restricted to XLA? And why? We are not yet sure that he is an XLA
and he has btk protein on WB.

Any comments, welcome!

Charlotte   


maryellen.conley


> Hi CCR,

> 

> Steve Holland and I have been interested in the

> Camplobacter/Flexispora/Helicobacter for a while.  I sent him an email last

> night to ask how many he had seen.  He said 4 or 5 but he commented on the

> difficulty in culturing these organisms and sometimes (like Paul's case) the

> patient is treated even without a postive culture. In addition to Paul's

> patient, I know about the 2 patients with XLA reported by the NIH- Clin Imm

> 97:121 (2000).  I have sent 2 more XLA patients to Steve and I was asked about

> another XLA from Asia with what sounds like the same.  Plus Stuart's case

> makes 6 cases.  There are several case reports in the literature.  In my

> experience, the patients are not acutely ill at all.  No fever, no elevated

> WBC but the symptoms may be quite variable.  Was assumed to be deep vein

> thrombosis in one patient; sometimes cellulitis, sometimes macules. Over time

> the ESR goes up and the Hgb goes down.  It may be that the duration of therapy

> should be influenced by the duration of symptoms before treatment.  One of the

> patients from NIH and one of my old patients had symptoms for a very long time

> (years) before the onset of treatment and one of the patients is still not

> under control (after nearly 20 years).  The other had a relapse after 6 months

> of IV therapy.  That's where the very aggressive approach came from. There are

> some published data indicating that both IgM and complement may play a role in

> clearing these organisms.  Steve has used plasma therapy in the recalcitrant

> patients with good success.  I would start with meropenem IV tid plus

> azithromycin and levaquin.

> 

> 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 Charlotte

> Cunningham-Rundles at mssm.edu

> Sent: Tuesday, July 26, 2011 9:02 AM

> To: PAGID

> Subject: Re: [CIS-PAGID] FW: XLA / helicobacter. .

> 

> 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-run

>> dles 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

>> 

>> 

>> ________________________________

>> Electronic mail from Children's Mercy Hospitals and Clinics. This

>> communication is intended only for the use of the addressee. It may

>> contain information that is privileged or confidential under

>> applicable law. If you are not the intended recipient or the agent of

>> the recipient, you are hereby notified that any dissemination, copy or

>> disclosure of this communication is strictly prohibited. If you have

>> received this communication in error, please immediately forward the

>> message to Children's Mercy Hospital's Information Security Officer

>> via return electronic mail at informationsecurityofficer at cmh.edu and

>> expunge this communication without making any copies. Thank you for your

>> cooperation.

> 

> 

> 

> Email Disclaimer:  www.stjude.org/emaildisclaimer

> 


Charlotte Cunningham-Rundles, MD, PhD
Departments of Medicine and Pediatrics
The David S Gottesman Professor
The Immunology Institute
Mount Sinai School of Medicine
1425 Madison Avenue
New York, NY 10029
Phone: 212 659 9268
Fax: 212 987 5593
Email: Charlotte.Cunningham-Rundles at mssm.edu