Thymus Transplantation With Immunosuppression

Inclusion Criteria

• •Transplant Inclusion
• •No age limit
• •Thyroid studies must be done and if abnormal, must be on therapy
• •DiGeorge diagnosis - must have 1 symptom from the following list:
• •Heart defect
• •Hypocalcemia requiring replacement
• •22q11 hemizygosity
• •10p13 hemizygosity
• •CHARGE association
• •Abnormal ears plus mother with diabetes (type I, type II, or gestational)
• •Atypical Diagnosis:
• •Must have, or have had, a rash. If rash present, biopsy of rash must show T cells in skin. If rash \& adenopathy resolved, must still have oligoclonal T cells.
• •Within 1 month of tx must have PHA response \>20 fold above background or \>5,000 cpm, whichever is higher, or response can be \< this.
• •Circulating CD3+ T cells \>50/mm3 but CD45RA+CD62L+CD3+ T cells \<50/mm or \<5% of CD3 count, whichever is higher (must be done 2x)
• •Immunoscope with \>40% oligoclonal TCRBV families. A 2nd test per sponsor discretion if T cell numbers increase or activation status changes.
• •If TREC done pre-tx must have TRECs \<100 per 100,000 CD3+ cells.
• •Typical Diagnosis:
• •Circulating CD3+ CD45RA+ CD62L+ T cells and \<50/mm3 or \<5% of total T cells
• •PHA response \>20 fold above background or \>5,000 cpm, whichever is higher.
• •2 studies must show similar immunological findings qualify for this study.
• •TRECs, if done, should be \<100/100,000 CD3 cells
• •Transplant Exclusion:
• •Heart surgery \<4 weeks pre-tx date
• •Heart surgery anticipated w/in 3 months of proposed tx
• •Rejection by surgeon or anesthesiologist as surgical candidates
• •Lack of sufficient muscle tissue to accept 0.2 grams/kg transplant