[PAGID] rituximab vs PID

[Kathleen E.Sullivan]

I have seen results of T cell studies in lupus patients after multiple 
courses  of rituximab and there was no effect on T cell numbers so I 
would be reluctant to ascribe the effect to rituximab.  I have a few 
ideas in terms of his process that perhaps you have already addressed.  
He can't really have SCID and be 11 years old so I presume you are 
asking whether he could  have such significant T cell dysfunction that 
he warrants a transplant.  I would wager that some degree of T cell 
dysfunction is as a consequence of his fungus and you will see some 
modest improvement over time.  Hard to believe he was born with this 
degree of compromise and has had only AHA until now.   I know you have 
tested for CGD but the NBT is not sensitive for mild mutations and CGD 
patients can have low T cell numbers and function that can worsen after 
fungal infections.  CGD would tie a lot of the features together so it 
might be worth retesting with a more sensitive fluorescent assay.  My 
last thought is whether he has had much adenopathy and should be tested 
for ALPS.   The caspase deficient forms can look a lot like CVID and 
have some unusual infections and could go along with AHA.

On Aug 15, 2005, at 4:12 PM, Rhoda Kagan wrote:

> Any suggestions as to the  management or diagnosis of this child are
> welcome!
>
>
>  11 yo Asian boy  with invasive murcormycosis following a course of
> rituximab for refractory autoimmune hemolytic anemia.  Subsequent 
> immune
> work up noted T cell lymphopenia and poor T cell function.  Is this a
> result of rituximab? If so, should we anticipate some improvement (it 
> has
> now been a year since last dose!) or did he have an undiagnosed PID 
> without
> any significant infections until last year?? Our questions are 
> twofold: 1.
> must he continue on anti-fungal treatment indefinitely (our ID 
> colleagues
> have suggested that he should); 2. should we consider BMT if he is 
> SCID?
>
> History:
>      -Born to non-consanguineous parents
>      - Thal Major dx'd at  8 mos;  autoimmune hemolytic anemia 
> diagnosed in
>      the first year of life
>      -transfusion-dependent with multiple transfusion reactions, 
> including
>      severe hemolysis, (Anti-Lu a Ab positive), initially managed with
>      pre-treatment  solucortef
>      *2003, hemolysis worsened;transfusion requirement
>      increased?..Prednisone commenced 10-40 mg qd
>      *2004, rituximab  (total 3 doses). Last dose july 2004 & IVIG q 4
>      weeks
>
>      -Sept. 2004: Mucormycosis infection of maxillary, ethmoid, and
>      sphenoid sinuses as well as peri-orbital region, treated with:
>      local and systemic amphotericin,  posaconazole, local irrigation 
> and
> regular debridement, hyperbaric O2 (38 courses),  and G-CSF
>
>      -Current management/disease status (presently radiographically 
> disease
>      free)
>      Posaconazole,  Prednisone , Septra prophylaxis, transfusions as
> required
>
> Immunology Work up  (all investigations done post-rituximab and 
> unchanged
> on repeat testing)
> HIV negative
> NBT, complement:  normal
>
> CBC: WBC 3.6  Hgb 63 Plt Nl  ANC 2.3 ALC 0.8
> CD3 0.487 CD19 0.639  CD4 0.070  CD8  0.320 NK 0.139
> PHA SI 1.22
> IgG 9.82 (on IVIG); IgM 2.10 IgA 2.26
>
>
> Rhoda Kagan, MD
> Montreal Children's Hospital
> rhoda.kagan at muhc.mcgill.ca
>
>
>
>
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Kathleen E. Sullivan MD PhD
Associate Professor of Pediatrics
The Children's Hospital of Philadelphia
(p) 215-590-1697
(f) 215-590-3044