Pyruvate Kinase Deficiency Medication

Updated: Feb 22, 2019
  • Author: Hassan M Yaish, MD; Chief Editor: George T Griffing, MD  more...
  • Print

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.

Large doses of salicylates should be avoided in patients with severe anemia, because these inhibit oxidative phosphorylation, thereby causing further ATP depletion.

Presurgical vaccines

Vaccines used prior to splenectomy include the following:

  • Polyvalent polysaccharide pneumococcal vaccine: Administered 1-2 weeks before splenectomy in patients over age 2 years

  • Conjugated pneumococcal vaccine: Administered to patients under age 2 years, although such patients are rare

  • H influenzae type b vaccine: The conjugate form is usually administered to children at age 2, 4, and 6 months


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.



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.



Class Summary

Polyvalent pneumococcal vaccine (PPV-23) protects against 23 serotypes of S pneumoniae; approximately 70% of invasive diseases caused by S pneumoniae result from these serotypes. This vaccine should be administered 1-2 weeks prior to splenectomy, to prevent or minimize future complications.

Pneumococcal 13-valent conjugate vaccine (PCV-13) protects against the 13 serotypes of S pneumoniae that cause the most severe pneumococcal infections in children. It replaced an earlier vaccine, PCV-7. [36, 37]

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.