Medication Summary
As in all persons with hemolytic anemias and because of the severe demand for folic acid, the potential for developing megaloblastic anemia in patients with pyruvate kinase deficiency can be prevented by administering supplemental folic acid. Packed red blood cell transfusion is reserved for persons who develop significant anemia.
Mitapivat, a first-in-class pyruvate activator, is approved to treat adults with hemolytic anemia associated with pyruvate kinase (PK) deficiency, regardless of transfusion status.
Vaccination against encapsulated bacteria—pneumococcus, meningococcus, and Haemophilus influenzae—is indicated in patients who undergo splenectomy. If a pediatric patient has not already received these vaccines as part of routine care, they should be administered at least 14 days before or after splenectomy. Adults may require boosters. [45]
Vitamins, Water-Soluble
Class Summary
Folic acid is used extensively in individuals with hemolytic anemia. Megaloblastic anemia may develop if folic acid is not supplied.
Folic acid (FA-8)
Folic acid is an important cofactor for enzymes used in the production of red blood cells.
Pyruvate Kinase-R Activators
Class Summary
Mitapivat, a first-in-class pyruvate kinase (PK) activator, improves hemoglobin values and reduces transfusion burden in patients with hemolytic anemia associated with PK deficiency by targeting the underlying defect. [10]
Mitapivat (Pyrukynd)
PK activator that acts by allosterically binding to pyruvate kinase tetramer and increasing PK activity. Red blood cell (RBC) form of pyruvate kinase (PK-R) is mutated in PK deficiency, which leads to reduced adenosine triphosphate, shortened RBC lifespan, and chronic hemolysis. Indicated for treatment of hemolytic anemia in adults with PK deficiency.
Antibiotics
Class Summary
Patients who undergo splenectomy are prone to fulminating infections with encapsulated organisms, most of which are sensitive to penicillins. Some clinicians recommend administration of prophylactic penicillin for 2-3 years following the procedure, while others recommend administration of prophylactic penicillin for life. Administer erythromycin instead if the child is sensitive to penicillin.
Penicillin VK
Penicillin VK inhibits the biosynthesis of cell wall mucopeptide.
Erythromycin (E.E.S., PCE, Ery-Tab, Erythrocin)
This antibiotic inhibits bacterial growth, possibly by blocking the dissociation of peptidyl transfer ribonucleic acid (tRNA) from ribosomes, causing RNA-dependent protein synthesis to arrest.
Vaccines
Class Summary
Patients who undergo splenectomy require vaccination against encapsulated bacteria, including Streptococcus pneumoniae (pneumococcus), Neisseria meningitidis (meningococcus), and Haemophilus influenzae.
Pneumococcal vaccine polyvalent (Pneumovax-23)
PPV-23 is used for prophylaxis against infection with S pneumoniae. It is employed in populations at increased risk of pneumococcal pneumonia (ie, >55 y, chronic infection, asplenia, immunocompromise). It contains capsular polysaccharides of pneumococcal types 1, 2, 3, 4, 5, 6B, 7F, 8, 9N, 9V, 10A, 11A, 12F, 14, 15B, 17F, 18C, 19F, 19A, 20, 22F, 23F, and 33F.
Pneumococcal 13-valent conjugate vaccine (Prevnar 13)
Pneumococcal 13-valent conjugate vaccine promotes active immunization against invasive disease caused by S pneumonia. It is a sterile solution of saccharides of capsular antigens of S pneumoniae serotypes 1, 3, 4, 5, 6A, 6B, 7F, 9V, 14, 18C, 19A, 19F, and 23F individually conjugated to diphtheria CRM197 protein.
Haemophilus influenza type b vaccine (ActHIB, Hiberix, PedvaxHIB)
This vaccine, which is used for routine immunization of children against invasive diseases caused by H influenzae type b, decreases nasopharyngeal colonization. The Centers for Disease Control and Prevention's (CDC's) Advisory Committee on Immunization Practices (ACIP) recommends that all children receive one of the conjugate vaccines licensed for infant use beginning routinely at age 2 months.
Conjugate forms are usually given in a series of 3 doses, at ages 2, 4, and 6 months. Children who have received primary vaccinations and a booster dose at age 12 months or older are usually protected and do not need further vaccination prior to splenectomy.
Meningococcal A C Y and W-135 diphtheria conjugate vaccine (Menactra, Menveo)
Meningococcal serogroup A, C, Y and W-135 capsular polysaccharide antigens individually conjugated to diphtheria toxoid protein carrier. Induces production of bactericidal antibodies directed against the capsular polysaccharides of serogroups A, C, Y and W-135, thus providing protection from invasive meningococcal disease.
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Peripheral blood smear in a child with splenectomy and pyruvate kinase deficiency.
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The Embden-Meyerhof pathway.
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Pyruvate kinase in the Embden-Meyerhof pathway.
