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