[PAGID] Pneumocystis in HIGM

[Cowan, Mort]

Why not transplant? Is there something else about this child that would
preclude considering this curative treatment?

Mort

Morton J. Cowan, M.D.
Professor of Pediatrics
Chief, BMT Division
UCSF Children's Hospital, Room M659
505 Parnassus Ave
San Francisco, CA 94143-1278
 
Phone: 415-476-2188
FAX: 415-502-4867
 

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-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Jack Bleesing
Sent: Friday, July 06, 2007 11:53 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] Pneumocystis in HIGM

I like a good discussion, gives me lots of "unclaimable" CME. 

Couple of points to keep this discusion going:

1. Lisa's patient seems to be one that will not become a patient
remembered for doing well, and where the discussion seems less about
prophylaxis and more about therapy. The point of other organisms is
certainly valid, adding to the list of factors on the "debit" side of
the list of outcome (compliance being an unfortunate other debit
factor).

2. I think it is human nature to remember the (few) patients, who seem
to be doing just fine (without BMT), while forgetting the patients who
didn't and/or who vanished of the radar screen (and have become
unknowns).

3. Putting a patient on IVIG and bactrim (whether for 5 years or for
life) cannot be the endpoints of standard-of-care therapy for XHIM (or
other immunodeficiency disorders with a compromised quantity and quality
of live).

Many regards and hoping you are enjoying the Summer months.

Jack



------------------------------------------------------------------------
---
Jack J.H. Bleesing, M.D., Ph.D.
Cincinnati Children's Hospital Medical Center
Division of Hematology/Oncology
3333 Burnet Avenue, MLC 7015
Cincinnati, OH 45229
513-636-4266 (phone)
513-636-3549 (fax)
Jack.Bleesing at CCHMC.org
http://www.cincinnatichildrens.org/immunodeficiencies/



>>> maryellen.conley at STJUDE.ORG 7/6/2007 11:09 AM >>>

Hi Lisa,
I would agree with Mel, I would worry about compliance.  I would also
want to make sure that he really was having PCP   pneumonia.  Maybe he
has steroid responsive pneumonitis.  I think you can have one or two
organisms on stain even when PCP is not the pathogen.  We used to see
that when transplant patients had CMV pneumonitis- you might find one
or
two PCP.

Your question brings up an issue that was discussed in summer school a
few years ago.  Do you need PCP prophylaxis in CD40L deficient
patients
once they are past 5 years of age?  Certainly the greatest
vulnerability
is in the first few years of life.  I keep the patients on prophylaxis
but I am a big believer in antibiotics.  Some of the other faculty did
not agree.  How old is your patient Lisa?  Do other people have young
adult patients with CD40L who have typical PCP?

I am not sure I agree with Jack Bleesing about stem cell transplant.
The prognosis of CD40L deficiency in the US seems to be better than in
Europe because we don't have as many problems with crypto.  We have
some
young adult CD40L deficient patients who are doing very well (even one
who is not very compliant).
Mary Ellen
 






Mary Ellen Conley, MD
Department of Immunology
St. Jude Children's Research Hospital
332 N. Lauderdale
Memphis, TN 38105-2794
FAX  901-495-3977
TEL  901-495-2576


-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org 
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Jack Bleesing
Sent: Friday, July 06, 2007 8:21 AM
To: lkobryn at emory.edu; pagid at list.clinimmsoc.org 
Subject: Re: [PAGID] Pneumocystis in HIGM

Keeping it short and to the point:

- BMT

- Agrressive treatment leading up and post BMT, including
anti-inflammatory therapy

- Evaluation and Propylaxis for co-morbid infections (viral, fungal,
etc)

I have battling PCP in a patient with XLP. It seemed in this patient
that his PCP wasn't as much an issue of failure of standard therapy as
an rather robust inflammatory/LPD component. Things got better on
optimized steroid therapy (after aggressively looking for other
organism).

J


>>> lkobryn at emory.edu 7/5/2007 11:39:20 PM >>>


I was wondering if anyone had experienced problems with failure of
bactrim prophylaxis in patients with hyperIgM.

One of our hyperIgM patients has had multiple episodes of pneumocystis
pneumonia despite reported compliance with daily bactrim for
prophylaxis. 
Pneumocystis
was isolated on BAL on at least one occasion and all episodes have
been
typical for pneumocystis pneumonitis and resolved with high dose
bactrim
and steroids. 
He has been tried on Atovoquone and failed this medication as well,
and
could not tolerate IV Pentamidine.

Has anyone seen failure of prophylaxis with Bactrim?  
What other prophylactic/therapeutic agents would you recommend?

Thanks

Lisa Kobrynski, MD, MPH
Assistant Professor of Pediatrics
Division of Allergy and Immunology
Emory University
404-727-3575
404-727-5045 (fax)