Adaptive Immunodeficiency in WHIM Syndrome
Marinos Kallikourdis
Round 1
Reviewer 1 Report
This review is exceptionally well-written, as it manages to thoroughly and systematically cover not only the entire spectrum of studies on the WHIM syndrome, but also those aspects of (wild-type) CXCR4 biology that are of relevance for setting the findings on the WHIM syndrome in context. As the studies on CXCR4 biology are extensive, the distillation of the insight relevant for WHIM is no easy task, but the authors have succeeded in doing so, in a manner that is very pleasant to read.
I have no requests or suggestions for improvements, though perhaps the authors could speculate on possible explanations for the finding that the adaptive immunity defects in the WHIM syndrome vary from patient to patient.
Reviewer 2 Report
The review by Majumdar and Murphy covers very interesting aspects on the impact of WHIM syndrome on adaptive immunity. In particular the authors point their attention on alteration occurring to lymphoid organs and to the different lymphocytes subsets that have been described in WHIM patients. A final focus on new therapeutic approaches is also included in the review.
The manuscript is well written and covers the most relevant literature on this topic.
