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X-linked hypogammaglobulinaemia (Bouton's disease)

defect in differentiation of pre-B to B cells (btk gene)
profound reduction in all immunoglobulin classes
B cells and plasma cells reduced
T-cells normal
presentation
- 3-6 months (maternal IgG levels declining
- infections
  - meniningitis
  - mycoplasma
Treatment
- immunoglobulin replacement

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