In boys with<unk> -linked<unk>  disorder, the inability to mount an immune response to EBV may lead to death via hemophagocytic lymphohistiocytosis (HLH). Patients may also develop dysgammaglobulinemia and malignant non-Hodgkin lymphoma, even without exposure to EBV. Other observed symptoms of<unk>  include aplastic anemia, vasculitis, chronic gastritis, and skin lesions, as well as IM. Nearly half of<unk>  patients express humoral immune anomalies, which can include diminished responses to vaccines and low levels of immunoglobulin G (IgG). Patients produce insufficient numbers of CD27 memory B cells.
X-linked lymphoproliferative disease
Autoimmune lymphoproliferative syndrome
Adult-onset immunodeficiency syndrome
Primary immunodeficiency
RAS-associated autoimmune leukoproliferative disorder
X-linked severe combined immunodeficiency
Nodal marginal zone B cell lymphoma
Splenic marginal zone lymphoma