Immunization of children and adolescents with primary and secondary immunodeficiencies

Category	Example of specific immunodeficiency	Vaccine contraindications	Effectiveness and comments, including risk-specific vaccines*
Primary^¶
B cell (humoral)	Severe antibody deficiencies (eg, X-linked agammaglobulinemia and common variable immunodeficiency)	OPV^Δ, BCG, smallpox, LAIV, YF, and live-bacteria vaccines^◊; no data for rotavirus vaccines	Effectiveness of any vaccine is uncertain if dependent only on humoral response (eg, PPSV23 or MPSV4); IVIG therapy interferes with response to all vaccines, therefore, annual IIV is the only vaccine given to patients receiving IVIG therapy; routine inactivated vaccines can be given if not receiving IVIG.
B cell (humoral)	Less severe antibody deficiencies (eg, selective IgA deficiency and IgG subclass deficiencies)	OPV^Δ, BCG, YF vaccines; other live vaccines^§ appear to be safe	All vaccines probably are effective; immune response may be attenuated; vaccines should be given as on the annual immunization schedule for immunocompetent people.
T cell (cell mediated and humoral)	Complete defects (eg, severe combined immunodeficiency, complete DiGeorge syndrome)	All live vaccines^◊^§^¥	All inactivated vaccines probably are ineffective. The only vaccine that should be given if the patient is receiving IVIG is annual IIV if there is some residual antibody production.
T cell (cell mediated and humoral)	Partial defects (eg, most patients with DiGeorge syndrome, hyperimmunoglobulin M syndrome, Wiskott-Aldrich syndrome, ataxia-telangiectasia)	All live vaccines^◊^§	Effectiveness of any inactivated vaccine depends on degree of immunosuppression. Routine inactivated vaccines should be given. Those with ≥500 CD3+ T lymphocytes/mm³, ≥200 CD8+ T lymphocytes/mm³, and normal mitogen response should receive MMR and VAR vaccine (but not MMRV). PPSV23 in addition to PCV13 is indicated in ataxia-telangiectasia.
Complement	Persistent complement component, properdin, or mannose-binding lectin (MBL) deficiency	None	All inactivated and live-virus vaccines on the annual immunization schedule are safe and probably are effective; PPSV23 at two years or older and an MCV4 vaccine are recommended in addition to standard vaccines.
Phagocytic function	Chronic granulomatous disease, leukocyte adhesion defects, myeloperoxidase deficiency	Live-bacteria vaccines^◊ OPV^Δ, smallpox, BCG, combined MMRV, LAIV^§; withhold MMR and varicella in highly immunocompromised children; YF vaccine may have a contraindication or precaution depending on indicators of immune function^‡	All inactivated vaccines are safe and probably are effective^†; live-virus vaccines probably are safe and effective.
Secondary*
	HIV/AIDS		Rotavirus vaccine is recommended on standard schedule; MMR and VAR are recommended for HIV-infected children who are asymptomatic and are not highly immunocompromised**^,¶¶; all inactivated vaccines, including IIV, may be effective; PPSV23 is recommended in addition to the standard PCV13 vaccine; consider Hib vaccine if not administered during infancy and MCV4 at 2 through 10 years of age.
	Malignant neoplasm, transplantation, autoimmune disease, immunosuppressive or radiation therapy	Live-virus and live-bacteria vaccines, depending on immune status^◊^§	Effectiveness of any vaccine depends on degree of immunosuppression; annual IIV is recommended unless receiving intensive chemotherapy or on anti-B cell antibodies; inactivated standard; PPSV23 is recommended at least eight weeks after last dose of PCV13. Vaccines are indicated if not highly immunosuppressed, but doses should be repeated after chemotherapy ends.
	Asplenia (functional, congenital anatomic, surgical)	None	All standard vaccines are safe and likely effective; PPSV23 at 24 months or older; MCV4-D (Menactra) should not be used before four weeks after completion of the four-dose series of PCV13 (eg, 22 months of age or older), because of interference with antibody response to PCV13 when vaccines are administered concurrently; other MCV4 vaccines are used in infants and toddlers as licensed by age without concern for interference with PCV13. In addition to standard vaccines, consider Hib vaccine if not administered during infancy.
	Chronic kidney disease	LAIV	All standard vaccines are indicated; PPSV23 is recommended at 24 months or older in addition to standard PCV13.
	CNS anatomic barrier defect (cochlear implant, congenital dysplasia of the inner ear, persistent CSF communication with naso-/oropharynx)	None	All standard vaccines are indicated; PCV13 if not received as standard; PPSV23 at two years or older (≥8 weeks after receipt of PCV13).

OPV: oral poliovirus; BCG: Bacille Calmette-Guérin; LAIV: live-attenuated influenza vaccine; YF: yellow fever; PPSV23: 23-valent pneumococcal polysaccharide vaccine; MPSV4: quadrivalent meningococcal polysaccharide vaccine; IVIG: intravenous immune globulin; IIV: inactivated influenza vaccine; IgA: immunoglobulin A; IgG: immunoglobulin G; IgM: immunoglobulin M; MMR: measles-mumps-rubella; VAR: varicella vaccine; MMRV: measles-mumps-rubella-varicella; PCV13: 13-valent pneumococcal conjugate vaccine; MCV4: quadrivalent meningococcal conjugate vaccine; HIV: human immunodeficiency virus; AIDs: acquired immunodeficiency syndrome; Hib: Hemophilius influenzae type b; CNS: central nervous system; CSF: cerebrospinal fluid; cART: combination antiretroviral therapy.
* Other vaccines that are recommended universally or routinely should be given if not contraindicated.
¶ All children and adolescents should receive an annual age-appropriate inactivated influenza vaccine. LAIV is indicated only for healthy people 2 through 49 years of age.
Δ OPV vaccine no longer is available in the United States.
◊ Live-bacteria vaccines: BCG and Ty21a Salmonella typhi vaccine.
§ Live-virus vaccines: LAIV, MMR, VAR, MMRV, herpes zoster (HZV), OPV, YF, vaccinia (smallpox), and rotavirus.
¥ Regarding T-lymphocyte immunodeficiency as a contraindication to rotavirus vaccine, data only exist for severe combined immunodeficiency syndrome.
‡ YF vaccine is contraindicated in HIV-infected children younger than six years who are highly immunosuppressed. There is precaution for use of YF vaccine in asymptomatic HIV-infected children younger than six years with total lymphocyte percentage of 15 to 24% and older than six years with CD4+ T-lymphocyte counts of 200 to 499 cells/mm³.^[1]
† Additional pneumococcal vaccine is not indicated for children with chronic granulomatous disease beyond age-based standard recommendations for PCV13, because these children are not at increased risk for pneumococcal disease.
** HIV-infected children should receive immune globulin after exposure to measles and may receive varicella vaccine if CD4+ T-lymphocyte count ≥15% of expected for age for those younger than five years, or CD4+ T-lymphocyte count ≥200 cells/mm³ for those five years or older.
¶¶ People with perinatal HIV infection who were vaccinated with a measles, rubella, or mumps-containing vaccine prior to the establishment of cART should be considered unvaccinated and should receive two appropriately spaced doses of MMR vaccine once effective cART has been established (at least six months with CD4+ T-lymphocyte percentage ≥15% for children younger than five years or CD4+ T-lymphocyte count ≥200 cells/mm³ for children five years or older).

Reference:

Centers for Disease Control and Prevention. Yellow fever vaccine: Recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Recomm Rep 2010; 59:1.

From: Kimberlin DW, Brady MT, Jackson MA, Long SS (Eds). Red Book: 2015 Report of the Committee on Infectious Diseases, 30th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2015: 74-89. Copyright © 2015 American Academy of Pediatrics. Reproduced with permission.

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Immunization of children and adolescents with primary and secondary immunodeficiencies