[PAGID] rituximab vs PID

[Rhoda Kagan]

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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