Table 3.

Laboratory testing

Baseline and Post-Transplant Laboratory Monitoring
Time interval of evaluation: baseline; post-transplant at 3 months ± 2 weeks; 6 months ± 4 weeks; 12 months ± 4 weeks; years 2-5 post-transplant every 12 months ± 6 months; beyond five years post-transplantation every 3 years ± 1 year after the first five years.
Recommended Studies
Quantitative immunoglobulins	IgG, IgA, IgM with notation as to whether the patient is currently on IVIG and if so the     dose and date of last administration
Isoagglutinins	Anti-A and anti-B titers (include patient and donor blood type)
Immunization	Provide vaccine used, pre and post (include time following immunization) titers,     information regarding use of IVIG and if on IVIG replacement therapy,     provide timing of the pre and post titers relative to IVIG administration
Lymphocyte proliferation	Mitogen PHA: provide percent of normal response = the patient raw data cpm (or dpm) of     stimulated cells divided by the lowest cpm (or dpm) of the control (normal)     response established for the performing lab Other mitogens including CD3 can be reported but are not essential
	Antigen (if performed) Tetanus: provide percent of normal response = the patient raw data cpm (or dpm) of     stimulated cells divided by the lowest cpm (or dpm) of the control (normal)     response established for the performing lab and date of last tetanus     immunization Candida: provide percent of normal response = the patient raw data cpm (or dpm) of stimulated cells divided by the lowest cpm (or dpm) of the control (normal) response established for the performing lab
Flow cytometry	Testing for T cell and B cell surface antigens to be performed as follows; recommended to be performed centrally
	Surface antigens: The following should be evaluated at each interval and both percent     and absolute number should be reported: CD3, CD4, CD8, CD19 (or CD20),     CD3-/CD16/56
	Naïve T cells: CD4/CD45RA/CD45RO and CD8/CD45RA/CD45RO as three color     studies reporting CD4+/CD45RA+ and CD8+/CD45RA+ (additional markers     for naïve cells are not required but could be evaluated including CD27, CD31,     CD62L and CCR7)
	B cell subset: CD19/CD27/anti-IgD as a three color tube (report CD19+/CD27+/IgD+     and CD19+/CD27+/IgD−)
Thymopoiesis	TREC analysis: Guthrie card blood spot method will be performed centrally.
Chimerism	T cell, B cell and myeloid chimerism should be performed at 12 months and the method used should also be reported.
Genotyping	All patients not previously genotyped should have a genetic diagnosis established.
Disease-specific assay	The following examples are provided For SCID or CID: Expression of disease-specific proteins in different lineages     and at various developmental stages (eg, gamma chain in naïve vs.     memory B cells in patients with mixed chimerism) Expression of MHC II molecules in different lineages (for Bare     Lymphocyte Syndrome) For WAS: WAS protein levels For CGD: NADPH oxidase activity