[CIS PIDD] [cis-pidd] low CD4 counts after partial thymectomy in cardiac patient

[Yesim Yilmaz]

Hi Allyson,
This could still be a Di George patient without the deletion. I am following a similar patient with Tbx1 mutation. Does he have any facial dysmorphism ( posteriorly rotated ears may be easily missed), speech abnormality, sub mucosal cleft etc? 
All the best, 
Yesim 

Yesim Yilmaz Demirdag 
Columbia University Medical Center 
212-305-2300 

Sent from my iPad

> On Apr 22, 2015, at 3:48 PM, Larkin, Allyson <allyson.larkin at chp.edu> wrote:
> 
> Dear Colleagues,
> 
> 
> 
> I recently saw a 9 y/o male for a second opinion of lymphopenia. He has a hx of tetralogy of fallot (s/p repair and partial thymectomy). He had immune studies done when he presented with a year of sinus disease. CD3 and CD4 counts are low (CD3 346, CD4 193), CD19 and CD16 are normal. Proliferation is close to normal. Immunoglobulins and vaccine response are normal. FISH for 22q11 is normal.
> 
> 
> 
> I think it's likely that his counts could be low secondary to his partial thymectomy. He has never had fungal or opportunistic infections.
> 
> 
> 
> Has anyone ever followed similar patients and considered using pneumocystis prophylaxis in the setting of the low CD4 counts?
> 
> 
> 
> My understanding is that infection risk has been looked at in neonates who had thymectomy in the setting of surgical procedures for congential heart disease. In a three year f/u study, these children did well, despite profound lymphopenia, with no more serious infections than healthy controls. However, this has not been followed further out (Mancebo, E. et al. Longitudinal analysis of immune function in the first 3 years of life in thymectomized neonates during cardiac surgery. Clinical and Experimental Immunology, 154: 375-383).
> 
> 
> 
> Thanks in advance for your thoughts,
> 
> 
> 
> Allyson Larkin, MD
> 
> Assistant Professor of Pediatrics
> 
> Children's Hospital of Pittsburgh of UPMC
> 
> allyson.larkin at chp.edu<mailto:allyson.larkin at chp.edu>
> 
> 412-692-7885
> 
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