[CIS PIDD] [cis-pidd] Low T-cells post-Heart Transplant

Tue Apr 25 20:29:23 EDT 2017

Colleagues:

I follow a 23yo young man who was born with hypoplastic left heart.  He had the usual surgeries including a Fontan procedure.  He developed very severe protein-losing enteropathy (albumin levels ~2mg/dL).  By 17yo he had pan-hypogammaglobulinemia and T-lymphopenia (CD4 worse than CD8).  Despite the numbers he really did not experience severe, chronic or opportunistic infections.

He received a heart transplant in June 2016.

As expected his serum albumin has gradually improved - latest 3.6.  However, he still has profound pan-lymphocyte deficiency:  CD3+ 46% (97), CD4+ 9% (18), CD8+ 34% (70).

He is still on prednisone (5mg/day), tacrolimus and mycophenolate.

What is the likelihood that he will repopulate his lymphoid populations?

Joe Church
Children's Hospital Los Angeles

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