Affecting T cells B cells and neutrophils
An rare autosomal recessive or sometimes X-linked disorder where T 408 cells, B cells and granulocytes are absent. Such children present with serious infection at birth or shortly afterwards. They have no lymph nodes or tonsils, and the usual thymic shadow is absent. Bone marrow is hypoplastic, and there may also be thrombocytopenia and anaemia. It appears to be a pluripotential stem cell failure and carries a dire prognosis. The only curative therapy is BMT.