[CIS PIDD] [cis-pidd] Advice re: CD40L - should haploidentical donors be considered?

[CIS-PIDD]

Dear colleagues,

We would appreciate your advice with the following question:

•         In patients with mixed ethnicity with low likelihood of finding matched-related and matched-unrelated donors, when (if ever) would you consider moving to a T-cell reduced haploidentical donor?

A brief case history is below:

Our patient is a 4 yo M with confirmed XL-HIGM syndrome, who presented classically in early childhood with PJP pneumonia. He has been infection free with good growth for the past year and is maintained on weekly SCIG, G-CSF to maintain normal neutrophil count, TMP-SMX for pneumocystis prophylaxis, and cryptosporidium precautions. There is no evidence of lung or liver disease at present.

He is of mixed Asian and Caucasian background. A younger sib is not a match. After 5 searches we remain unsuccessful in finding a match, and suspect future searches will have low yield.

Thank you for your time – any feedback would be appreciated!


Dr. Victoria Cook MSc MD FRCPC
PGY-5, Allergy and Clinical Immunology
University of British Columbia
41-01536




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