Shingles Vaccines in Felty Syndrome with Severe Chronic Neutropenia
The live-attenuated zoster vaccine (Zostavax) is contraindicated in patients with Felty syndrome and severe chronic neutropenia, but the recombinant zoster vaccine (Shingrix) can be safely administered. 1
Vaccine Type Matters Critically
Live-Attenuated Zoster Vaccine (Zostavax)
Live viral vaccines should be administered to patients with congenital or cyclical neutropenia but are contraindicated in patients with undefined phagocytic cell defects. 1 Felty syndrome represents an acquired, chronic neutropenia associated with autoimmune disease and immunosuppression—not a simple congenital neutropenia. The key distinction is:
- Patients with congenital or cyclical neutropenia can receive live viral vaccines because their immune defect is limited to neutrophil production, with preserved lymphocyte function 1
- Patients with Felty syndrome typically have broader immune dysfunction including lymphocyte abnormalities, splenomegaly, and are often on immunosuppressive therapy for their underlying rheumatoid arthritis 2, 3, 4
- Live viral vaccines should not be administered to patients with any undefined phagocytic cell defect 1
The IDSA guidelines specifically state that live viral vaccines should not be given to highly immunocompromised patients, and Zostavax should not be administered to highly immunocompromised patients. 1
Recombinant Zoster Vaccine (Shingrix)
Shingrix is the preferred option because it is not a live vaccine and carries no risk of vaccine-strain infection. 1 This recombinant subunit vaccine using VZV glycoprotein E with adjuvant AS01B:
- Is safe in immunocompromised populations including stem cell transplant recipients and HIV-infected persons 1
- Does not pose risk of vaccine-related infection unlike the live-attenuated vaccine 1
- Is more immunogenic than live-attenuated vaccine even in immunocompetent adults 1
Clinical Context for Felty Syndrome
Patients with Felty syndrome face significant infection risk due to severe neutropenia, making them particularly vulnerable to herpes zoster reactivation. 2, 3, 4 The syndrome is characterized by:
- Severe chronic neutropenia with absolute neutrophil counts often <1,000/μL 2, 3
- Recurrent bacterial infections and sepsis as major causes of morbidity and mortality 2, 3, 4
- Underlying autoimmune disease requiring immunosuppressive therapy 2, 3
Practical Recommendations
Administer Shingrix (two doses, 2-6 months apart) to patients with Felty syndrome and severe chronic neutropenia. 1 This provides protection against herpes zoster without the safety concerns of live vaccination.
Absolutely avoid Zostavax in this population due to the risk of disseminated vaccine-strain VZV infection in the setting of severe immunocompromise. 1
Important Caveats
- If the patient is on low-dose immunosuppression only (methotrexate <0.4 mg/kg/week, azathioprine <3.0 mg/kg/day, or prednisone <20 mg/day), some guidelines suggest live-attenuated vaccine could theoretically be considered 1—however, given the severe chronic neutropenia in this case, Shingrix remains the safer choice
- Patients with Felty syndrome often require G-CSF therapy to manage neutropenia 2, 5, 3, but this does not change vaccine recommendations
- All inactivated vaccines should be administered per CDC schedule including pneumococcal vaccines (PCV13 followed by PPSV23), given the high infection risk 1