Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) is an immune-compromising illness, and infection is one of the most significant risks for those of us afflicted with this condition. Vaccinations play a vital role in the care of CLL/SLL patients to reduce the risk of contracting and suffering certain infectious diseases. This article aims to help provide guidance because knowing which vaccinations to get can be confusing. This article will lay out the current United States national recommendations to help you make the proper choices.
The guidelines to be presented below have been developed by the United States Advisory Committee on Immunization Practices (ACIP), a group of medical and public health experts from multiple medical specialties and public health agencies under the oversight of the United States Department of Health and Human Services. The ACIP extensively reviews the most current data. After intensive study and documentation, initial recommendations are drafted and then presented to the Centers for Disease Control (CDC) for review and final approval before being placed into national guidelines.
The complete CDC adult vaccination guidelines can be found here: https://www.cdc.gov/vaccines/schedules/downloads/adult/adult-combined-schedule.pdf
Pay special attention to Table 2, which deals with immunizations for the immunocompromised.
Guidelines may vary for those in other countries, and it may be best to check your national recommendations. The links to these sites can be found below.
For those of you in Britain: https://www.nhs.uk/conditions/vaccinations/
For those of you in Australia: https://www.health.gov.au/health-topics/immunisation/when-to-get-vaccinated/national-immunisation-program-schedule
Before delving into the individual vaccines, it is crucial to understand that vaccines are developed using different methodologies and can be divided into two classes: inactivated and live vaccines. An important point to always remember is that we should only receive inactivated vaccines and never a live vaccine produced with an attenuated (weakened) but living form of a virus. Due to our weakened immune systems, the injected virus potentially may reactivate and cause actual infection and illness. Examples of live vaccines to be avoided include:
- Intranasal Flu vaccine (FluMist)
- Measles, Mumps, Rubella (MMR)
- Zostavax (no longer used in the USA as of November 18, 2020)
- Yellow Fever
Moving onto the inactivated vaccines of which there are many available, it is important to note that they can be divided into those considered routine and those that are specialized. The specialized vaccines are beyond the scope of this article. For example, they are needed when traveling to a foreign country where diseases not commonly found in the US may be encountered. It is essential to know that some travel-related vaccines are live and may be contraindicated. The destination, duration of the trip, and other factors are used to determine which vaccines are indicated, and that information can be found through the CDC here: https://wwwnc.cdc.gov/travel
Below are the routine vaccines we should receive. Some of these vaccines require repeat dosing, and others do not. Indications and dosing schedules are outlined for you.
- Much has been written about the novel coronavirus, SARS-CoV-2, which has turned our world upside down since 2020. The illness is far-reaching with severe effects. Vaccination amongst other prevention strategies is of key importance, and extensive information about the virus can be found at https://cllsociety.org/covid-19/
- Influenza is a viral respiratory infection with a higher seasonal prevalence during the winter months and may infect the nose, throat, and lungs. It may cause pneumonia and even death in severe cases, so it is imperative to be vaccinated. In addition, because the virus may mutate and change from year to year and immune protection from a vaccine wanes over time, it is vital to get your vaccine annually.
- Get your vaccine soon upon its release, usually in early September.
- Avoid the live attenuated nasal vaccine, FluMist.
- There are many commercially available inactivated flu vaccines, all of which cover four strains of the virus, and the CDC does not recommend a particular formulation.
- However, one vaccine, Fluzone High Dose, contains a more significant amount of antigen than the other inactivated vaccines and may promote a more robust immune response. Therefore, it is approved by the Food and Drug Agency (FDA) for persons over age 65 and maybe a consideration.
- Because our immune systems are weak, we may not get a complete response to the vaccine, so it is crucial for our close contacts, who will likely have a more robust response, to be vaccinated to reduce the risk of passing the flu onto us.
- Pneumococcus is a bacterial infection that can cause severe illness in the form of pneumonia, blood infection, and meningitis. Persons at increased risk include the elderly and those of us with blood cancer. Over 90 serotypes of the pneumococcal bacteria and vaccines have been developed to target the types most at risk of causing severe disease.
- The recommendations for vaccination against pneumococcal disease were unchanged for many years. Still, with the release of new vaccines, significant changes have been made, with the most recent updates being released in January 2022. One goal of the latest recommendations has been to simplify the vaccination regimen. However, there remains some complexity which I will try to simplify in straightforward terms so you will know what vaccines you should get and when to get them.
- The best time to get vaccinated is early following your diagnosis of CLL/SLL, so do not delay.
- There are three currently available vaccines: Vaxneuvance (PCV15), Prevnar20 (PCV20), and Pneumovax 23 (PPSV23).
- The first two vaccines, PCV15, and PCV20, are conjugate vaccines (PCV) and have replaced the earlier versions: Prevnar 13 (PCV13 ) and Prevnar (PCV7).
- CLL/SLL patients who have not previously received a PCV or whose previous vaccination history is unknown should receive one dose of either PCV20 or PCV15. When PCV15 is used, it should be followed by a dose of PPSV23.
- When PCV15 is used, the recommended interval between administration of PCV15 and PPSV23 is ≥ one year. Still, a minimum interval of eight weeks can be considered for adults with an immunocompromising condition, including CLL/SLL.
- Special Circumstances:
- If previously you only received PPSV23, you may receive either PCV20 or PCV15 ≥ one year after the last PPSV23 dose. Another dose of PPSV23 is not then needed.
- If you previously received a PCV13, you should have a PPSV23 at least eight weeks later. However, if PPSV23 is not available, you may receive a PCV20.
- With the release of the updated guidelines in January 2022, the CDC no longer recommends a revaccination dose with the PPSV23 for patients with leukemia and lymphoma. Therefore, there are no recommendations for booster PCV15, PCV20, or PPSV23 vaccines. However, future directions may be forthcoming from the CDC.
- Shingles is a painful viral infection due to the reactivation of the chickenpox virus (Varicella), resulting in a blistering rash. One in three persons will develop shingles in their lifetime. The risk for shingles increases with advancing age and with weakened immune systems. A significant complication of shingles is postherpetic neuralgia which results in persistent pain following resolution of the rash and may occur in 10-18% of those who get shingles.
- There are two vaccines available, one called Zostavax, a live vaccine that should be avoided. Zostavax is no longer used in the USA but is available in some countries
- Shingrix, the inactivated vaccine, is the preferred vaccine and has been shown to reduce the risk of the painful blistering rash known as zoster and any persistent painful symptoms of postherpetic neuralgia. However, whether patients with CLL/SLL will respond to the vaccine as prior studies have shown a decreased response to the Influenza vaccine. Fortunately, two studies have shown an immune response to the Shingrix vaccine in both treatment naïve patients and those on Bruton tyrosine kinase inhibitors such as Ibrutinib. Therefore, one should not delay getting this vaccine if on treatment.
- Tetanus is a bacterial infection that causes muscle spasms, also known as “lock jaw.” The bacteria reside in the soil and may enter the body through an open wound. Diphtheriae is also a bacterial infection that usually spreads via respiratory droplets and causes an illness that generally affects the throat resulting in difficulty breathing but may also affect the skin. Pertussis, also known as whooping cough” is also transmitted by respiratory droplets and results in violent coughing that can be so severe as to result in fractured ribs. Tetanus and diphtheria are rarely reported in the United States though pertussis remains a more common infection. Tetanus, diphtheriae, and pertussis vaccination begins in childhood and requires repeated periodic vaccinations through adulthood.
- Tetanus vaccines are available in two forms:
- Tetanus, diphtheriae (Td), which protects against tetanus and diphtheriae.
- Tetanus, diphtheriae, pertussis (Tdap) protects against all three bacteria.
- A Td or Tdap routinely should be given every ten years. Previously, the CDC recommended a single Tdap in adulthood followed by periodic Td boosters. However, the CDC no longer recommends one vaccine over the other. However, a Tdap may be the better choice for a booster, especially if there will be close contact with an infant less than twelve months of age to prevent the risk of passing pertussis onto a child.
- A booster Td or Tdap may be indicated if five years have passed from the prior vaccine if there is a significant laceration. Again, either vaccine is acceptable, though a Tdap may be better.
Hepatitis B Vaccine
- Hepatitis B is a virus transmitted through exposure to blood and certain body fluids. It can cause severe acute liver inflammation and, in some cases, persist, causing chronic liver disease and eventually death. Vaccinations for hepatitis B are now routinely given starting at birth, but for those who did not get vaccinated, the vaccination series is recommended in adulthood.
- There are several hepatitis B vaccines available, all of which are equally recommended.
- In addition, there is a combination vaccine, Twinrix, containing both hepatitis A and hepatitis B vaccines combined. This may be a good choice as it would help protect against both viruses.
- When immunocompromise includes following a hematopoietic stem cell transplant (HSCT), serologic testing following the vaccine to test for an immune response may be indicated.
- Complete information can be found here: https://www.cdc.gov/mmwr/PDF/rr/rr5516.pdf
- In addition to the above routine vaccines recommended for all of us, a few selected vaccines are recommended when an additional underlying risk factor is present.
Hepatitis A vaccine is indicated when an additional risk factor is present, though optionally may also be given if desired.
- Hepatitis A is a viral infection of the liver and is usually transmitted through contaminated food and water. Hepatitis A is an uncommon infection in the United States and is more commonly found in less developed nations though sporadic outbreaks occur here. This vaccine is recommended for individuals with any of the following underlying risk factors: chronic liver disease, HIV, men who have sex with men, homelessness, travel to an area with high or intermediate endemic rates, injection or non-injection drug use, close personal contact with an international adoptee, work settings where exposure is more likely.
- The CDC database of travel destinations of high or intermediate endemic Hepatitis A rates can be found here: https://wwwnc.cdc.gov/travel/destinations/list
- The Hepatitis A vaccine is available as a stand-alone vaccine or combined form with the Hepatitis B vaccine. Speak to your physician about getting vaccinated if you have one of the above risk factors or will be traveling to a destination that puts you at risk of exposure to Hepatitis A or if you prefer to cover all bases and have this vaccine.
Haemophilus Influenzae Type B Vaccine
- Haemophilus influenzae type B is a bacterial infection that can cause a severe infection of the lung, brain, and bloodstream, not to be confused with influenza, a viral infection known as the flu. Vaccination to prevent this disease is generally given to children within the first year of life. In addition, the vaccine is recommended for certain adults, specifically those HSCT or those with an anatomically or functionally absent spleen. You should speak to your physician about vaccinations should you plan to undergo this procedure.
- For those with an anatomically or functionally absent spleen, one dose is recommended and, if possible, should be given at least 14 days before spleen removal.
- A 3-dose series four weeks apart starting 6-12 months after successful transplant should be given for those undergoing HSCT, even if previously vaccinated.
- Discuss with your healthcare team about Haemophilus Influenzae Type B vaccine
- Meningitis is a serious life-threatening illness causing inflammation of the layers surrounding the brain and spinal cord and blood stream infection. There are many different causes, some non-infectious and others infectious, including bacterial, viral, fungal, and parasitic agents. Vaccines have been developed to prevent a subset of bacterial meningitis. These vaccines are recommended for specific individuals, including adults who no longer have an anatomic or a functioning spleen.
- There are two commercially available meningitis vaccines:
- Meningococcal conjugate (MenACWY) vaccines
- Serogroup B meningococcal (MenB) vaccines
- Speak to your physician about the dosing and booster vaccination recommendations
In summary, there are many vaccines that we need and should be given early in our treatment. Unfortunately, we may not produce as strong an immune response to these vaccines as those with normal immune systems. Even so, we should not delay in getting vaccines to protect ourselves as best we can. Because of our unique situations, our treating physicians may choose to modify current vaccine recommendations, and it is best to follow your specialist’s advice. And it is essential to be aware that guidelines will likely change due to the rapid pace at which medical science is progressing. We will do our best to keep you updated.
I hope this has been helpful to guide you through yet another path along your CLL/SLL experience.
We share in this journey together.
Kim Davidson, MD
Kim Davidson received her Doctor of Medicine degree from the Medical College of Virginia. Following training in Obstetrics and Gynecology, and Family Medicine, she provided medical care and taught for over thirty years until recently retiring. She is a CLL patient and shares this journey with all of you.