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Hyperbilirubinemia
| Neonatal unconjugated hyperbilirubinemia may require treatment w/phototherapy &/or exchange transfusion, depending on level of bilirubin & age & weight of neonate. 1 Conjugated hyperbilirubinemia (obstructive jaundice) requires eval for cholestasis & appropriate treatment.
| Rarely, obstructive jaundice may develop in neonates due to excessive hemolysis → secondary liver damage (to date, not reported in EPB42-HS; more likely in severe hereditary hemolytic anemia); in such instances, consider overtransfusion to suppress endogenous erythropoiesis & interrupt continuing hemolysis & liver insult. 2 |
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Anemia
| Folic acid supplementation (400 µg 1x/d until age 1 yr; 1 mg 1x/d thereafter) Red blood cell transfusion as needed for hemolytic or aplastic crisis Routine immunizations (incl annual influenza vaccine) to prevent infections & precipitation of hemolytic or aplastic crisis
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Supplemental iron only in those w/confirmed iron deficiency Carefully monitor iron status w/ferritin & transferrin or TIBC saturation. Discontinue iron therapy after iron stores are repleted to avoid iron overload.
| Avoidance of iron supplementation unless concurrent iron deficiency is confirmed w/iron studies |
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Iron overload
| Strongly consider treatment w/iron chelator if child remains transfusion dependent after 1st yr of life. Monitor ferritin & obtain T2*-weighted MRI to determine hepatic iron levels if ferritin remains steadily ↑ (>300-500 ng/mL). | |
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Splenomegaly
| Immunizations recommended before splenectomy:
23-valent pneumococcal polysaccharide vaccine (PPSV23) for S pneumoniae given ≥2 wks before splenectomy Meningococcal conjugate vaccine for N meningitidis against serogroups A, C, W, & Y (MenACWY) given ≥2 wks before splenectomy Prevnar-13® H influenzae type b
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| Partial splenectomy is assoc w/lower risk for post-splenectomy sepsis & ↓ hemolysis; may be preferable for young children if done by experienced surgeon. | Antibiotic prophylaxis may be discontinued 1 yr after partial splenectomy if immune splenic function is adequate as assessed by pit count (% of pitted or pocked red cells). 4 |
| Splenectomy only in those w/severe disease & only after age 5 yrs | Rarely indicated in EPB42-HS, as disease severity is usually mild or moderate; splenectomy is curative but entails long-term risk for life-threatening infections. 5 |
Immune deficiency
(following splenectomy) | PPSV23 booster dose 5 yrs after 1st dose | No more than 2 doses of PPSV23 are recommended. 6 |
| Meningococcal conjugate vaccine booster dose:
| Serogroup B meningococcal vaccines recommended for those age ≥10 yrs w/history of splenectomy |
| Prophylactic antibiotics. Penicillin V-K 250 mg 2x/dy OR erythromycin for those allergic to penicillin | Controversy exists re duration of prophylactic antibiotics post splenectomy: some hematologists recommend for 3 yrs post splenectomy, others for life. 4 |
| Treatment of fever. Immediate medical attn & IV antibiotics w/good coverage for encapsulated organisms (typically ceftriaxone in doses adequate to treat meningitis: 100 mg/kg/d ≤2 g/d in single daily dose) | Incidence of post-splenectomy sepsis, a life-threatening complication, is higher than in general population. |
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Cholelithiasis
| Cholecystectomy
In those w/signs/symptoms of cholelithiasis In those w/history of cholelithiasis undergoing splenectomy Consider in asymptomatic persons when cholelithiasis is identified on screening ultrasound to prevent complications incl obstructive jaundice &/or pancreatitis.
| In children who require cholecystectomy, concurrent splenectomy is no longer recommended; need for splenectomy should be assessed on a case-by-case basis & the indication of splenectomy justified independently. 8 |
