Preimplantation genetic diagnosis for X-linked agammaglobulinemia: a case report

Fertil Steril. 2009 May;91(5):1958.e5-7. doi: 10.1016/j.fertnstert.2009.01.093. Epub 2009 Mar 6.

Abstract

Objective: To report the first clinical experience of preimplantation genetic diagnosis (PGD) for X-linked agammaglobulinemia (XLA), which resulted from a point mutation in Bruton's tyrosine kinase (BTK) gene.

Design: Case report.

Setting: Clinical IVF laboratory.

Patient(s): A couple carrying BTK gene mutation (c.832C>T) that is associated with XLA.

Intervention(s): Day 3 embryos, obtained by IVF with intracytoplasmic sperm injection, underwent blastomere biopsy and DNA testing via nest polymerase chain reaction and restriction enzyme analysis to detect the presence of a maternal BTK mutation.

Main outcome measure(s): Successful PGD for a BTK gene mutation and transfer of healthy embryos.

Result(s): The patient received two PGD cycles. In total, 19 oocytes were fertilized, 15 embryos were suitable for embryo biopsy, 6 were diagnosed as unaffected, 6 were carriers, 2 were mutant, and the remaining 1 did not provide any diagnosis. In two treatment cycles, two ETs of three embryos each were performed. Unfortunately no pregnancy was achieved.

Conclusion(s): We are the first to report a case of PGD for XLA. The result indicates that PGD is feasible for this genetic disease and may represent a viable alternative to prenatal diagnosis with the subsequent option of terminating an affected pregnancy.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Agammaglobulinaemia Tyrosine Kinase
  • Agammaglobulinemia / diagnosis*
  • Agammaglobulinemia / genetics
  • Female
  • Genetic Diseases, X-Linked / diagnosis*
  • Genetic Diseases, X-Linked / genetics
  • Humans
  • Point Mutation
  • Polymerase Chain Reaction
  • Preimplantation Diagnosis*
  • Protein-Tyrosine Kinases / genetics

Substances

  • Protein-Tyrosine Kinases
  • Agammaglobulinaemia Tyrosine Kinase
  • BTK protein, human