Primary
Primary
- Genetic defects – usually autosomal recessive:
- Function of cytotoxic T cells or NK cells
- Inflammasome regulation
Secondary (Acquired)
Secondary (Acquired)
Viral Infections
- EBV
- HSV
- HIV
- CMV
29%
Other Infections
- Bacterial
- Protozoal (Malaria, Leishmania)
- Fungal (Candida, Aspergillus)
- Mycobacteria
- Mycoplasma
20%
Malignancies:
- Natural killer (NK) cell lymphomas
- B-cell lymphomas
- Hodgkin lymphoma
- Leukaemia
- Other hematologic neoplasms
- Solid tumours
27%
Rheumatological Conditions
- Juvenile idiopathic arthritis
- SLE
- Adult-onset Still's Disease
- Rheumatoid Arthritis
7%
Immune Deficiency States
- Stem or bone marrow transplant
- Solid-organ transplant
- Severe combined / common variable immunodeficiency
6%
Other
- Haemodialysis
- Pregnancy
- Vaccination
Rare