Salient aspects of management and prognosis of ALPS
| Diagnosis and genetic counseling at presentation |
| Significant number of ALPS patients do not need any intervention for asymptomatic lymphadenopathy and splenomegaly that often seems to get better with age. |
| Use of spleen guards made of thermoplastic material for protecting enlarged spleens from trauma |
| Avoid splenectomy |
| For unavoidable surgical asplenia: use 13 valent conjugate and 23 valent polysaccharide vaccines against pneumococcal sepsis |
| Med alert bracelet, fever alert, and long-term antibiotic prophylaxis |
| Autoimmune cytopenias: short-term steroids and IVIG |
| Steroid-sparing measures: mycophenolate mofetil and sirolimus |
| Vigilance for lymphoma: role of periodic CT and FDG-PET scans |
| Deaths (13 of 257 patients) in our cohort: death resulting from sepsis with asplenia (9), malignancies (4) |
| Diagnosis and genetic counseling at presentation |
| Significant number of ALPS patients do not need any intervention for asymptomatic lymphadenopathy and splenomegaly that often seems to get better with age. |
| Use of spleen guards made of thermoplastic material for protecting enlarged spleens from trauma |
| Avoid splenectomy |
| For unavoidable surgical asplenia: use 13 valent conjugate and 23 valent polysaccharide vaccines against pneumococcal sepsis |
| Med alert bracelet, fever alert, and long-term antibiotic prophylaxis |
| Autoimmune cytopenias: short-term steroids and IVIG |
| Steroid-sparing measures: mycophenolate mofetil and sirolimus |
| Vigilance for lymphoma: role of periodic CT and FDG-PET scans |
| Deaths (13 of 257 patients) in our cohort: death resulting from sepsis with asplenia (9), malignancies (4) |