• Avoid contact sports and prevent head injuries.
• Avoid crowds.
• No live-virus vaccinations

Patient Monitoring As needed for therapy; monitor for infections, for progression of disease, and for complications

No special diet is required.

• Patient/parent counseling to cope with disease and outcome
• Genetic testing and counseling for family

• Usual course is acute and chronic infections with progressive decrease in immune status.
• Survival to adulthood, formerly rare, is now increasing owing to advances in medical treatment. HSCT is potentially curative; it can restore platelets and immune function.
• Average life expectancy is 11 years, with some living past 20 with HSCT.
• Causes of death include infection, bleeding, and malignancy.

• Severe infections, especially after splenectomy:
  • Antibiotic prophylaxis, IVIG replacement
  • Pneumocystis jiroveci prophylaxis with trimethoprim–sulfamethoxazole or pentamidine
• Hemorrhage, cerebral (common):
  • Avoid circumcision of at-risk newborns who have thrombocytopenia.
  • Avoid medications that interfere with platelet function.
• Malignancies (lymphoreticular, lymphoma): 13% develop lymphoma at an average age of 9.5 years
• Autoimmune disease in 40% of those who survive infancy; can be aggressive:
  • Hemolytic anemia
  • Immune thrombocytopenic purpura
  • Immune-mediated arthritis
  • Vasculitis