Reticular dysgenesis

Reticular dysgenesis is a very rare autosomal recessive form of SCID, which generally leads to early death. The disorder usually manifests early in the neonatal period, with the sign of sepsis as the first day of life, mainly caused by severe leukopenia. The disease is characterized by lack or very reduced levels of B and T cells, thrombocytes involved in blood clotting, and granulocytes that form the majority of blood leukocytes, due to the failed maturation of not only lymphoid but also of myeloid precursor cells.

Therapeutic options