Childhood Cancer

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Childhood Cancer Survivors

Spleen and lymphatic system

The spleen is part of the lymphatic system—a body-wide network of vessels and organs. The tonsils, thymus, and spleen are organs composed of lymphoid tissue. The tonsils, located in the back of the throat, filter and destroy bacteria. The thymus, a small organ beneath the breastbone, plays a role in helping white blood cells mature. The spleen is an organ in the upper abdomen that removes old red blood cells and platelets from the blood. It also stores red blood cells and performs other important functions of the immune system. Figure 16-1 shows the various parts of the lymphatic system.

The lymphatic system

Figure 16-1. The lymphatic system

Organ damage

Much is still not known about immune status and risk of infections in childhood cancer survivors. By far, the best-documented threats to the body’s immune system are removal of the spleen or high-dose (4000 centigray [cGy] or more) radiation to the spleen. Both of these treatments have been used to treat thousands of children and teens with Hodgkin lymphoma. Patients with non-Hodgkin lymphoma are also sometimes treated with high-dose radiation to the spleen. In this book, the term “asplenia” refers to either the absence of the spleen or a spleen that no longer functions after high-dose radiation.

I had 4400 rads (cGy) of mantle radiation, 4400 to the mediastinum, and 4400 to the diaphragm and spleen area in 1968 to treat my Hodgkin’s stage IIA. CT (computed tomography) scans show no evidence of a spleen. My immune system still works okay, so maybe whatever’s left of the spleen still supplies some service. I’m glad to still have it. The kidney on my left side is atrophied as well. I have lots of other problems, but my immune system has held up.

Susceptibility to infection is also a problem for survivors treated for chronic graft-versus-host disease (GVHD) after a stem cell transplant (e.g., bone marrow, cord blood, or peripheral blood). 1 The doses of total body radiation used to prepare children for transplantation do not destroy the spleen, although they do destroy bone marrow function. This is discussed later in the Bone marrow section of this chapter.

Signs and symptoms

Susceptibility to viral and bacterial infections is the hallmark of survivors with lowered immunity due to asplenia. These infections can progress rapidly and, in some cases, are life-threatening. Signs and symptoms of infection are as follows:

  • Fever

  • Sore throat

  • Cough

  • Shortness of breath

  • Enlarged lymph glands

  • Fatigue

  • Chills

  • Red vesicles that break open, then crust over (i.e., chicken pox)

  • Red vesicles that travel in lines along the paths of nerves (i.e., shingles)

Ten years ago, I had a splenectomy, 2500 and 2800 cGy of mantle radiation, and ABVD (combination of four chemotherapy drugs) to treat my Hodgkin’s. I get sick frequently. So far, I have not had overwhelming sepsis, but I have had many illnesses that required daily communication with the doctor to monitor them. During my illnesses, I spend from 1 to 3 weeks in bed, and it’s about a month before I’m back to full energy level. I always know when something is starting because I get a weird feeling. I feel shaky, slightly short of breath, and my balance is off. Within a day or two, the sore throat, fevers, and severe illness start. I’ve learned to start the antibiotics when the feeling starts, and then the illness has a shorter course. It happens from two to four times a year.

Screening and detection

Survivors without spleens or with nonfunctional, irradiated spleens need an annual exam that includes a detailed history of infections and illnesses. Methods to screen for and detect infections that require antibiotics and/or hospitalization should be part of a plan that you and your healthcare provider work out.

Medical management

Survivors with asplenia are at increased risk for bacterial infections that overwhelm the immune system very quickly. Infections with certain types of bacteria can become life-threatening in a matter of hours. 2 For this reason, children with asplenia are given daily preventive penicillin (or erythromycin if they are allergic to penicillin) and their parents are told to call the doctor immediately if the child develops a fever of 101°F (38.3°C) or higher. The Children’s Oncology Group’s (COG’s) guidelines recommend that survivors with a temperature above 101°F (38.3°C) be given a long-acting, broad-spectrum antibiotic (e.g., ceftriaxone) and be closely monitored while awaiting the results of blood cultures. 3

If you are an adult survivor with asplenia, your follow-up program will likely instruct you to call your healthcare provider immediately if you develop a fever higher than 101°F (38.3°C). Because bacterial infections are very dangerous for survivors who have asplenia, you will be prescribed long-acting, broad-spectrum antibiotics even before tests show what type of organism your body is fighting.

Post-splenectomy infection risks are not commonly understood by the general public, and sometimes not even in the medical profession. My oncologist is fantastic as far as response to possible post-splenectomy sepsis, but I find that even some experienced physicians don’t get it. I’ve called in at my general practice doctor’s office before with chills and a temp of 102°—on prophylactic penicillin V, I might add—only to be told that: (a) they might be able to work me in about a week later if someone canceled; (b) no, I couldn’t speak with or leave a message for the doctor; (c) no, I couldn’t speak with or leave a message for his nurse; and (d) they were booked and no, they couldn’t possibly see me earlier than 1 week or more away. And that was my GP, whom I was supposed to call for that kind of thing.

It’s so much easier dealing with my late effects oncologist. I can call the office, tell the secretary who I am and what’s wrong, and she says she’ll page him to call me. I then actually get a quick callback, and he evaluates whether I need to come in or just do the antibiotics at home and come in if I get any worse. It really bothers me to have doctors who handle it otherwise because I’m very aware of how lethal this can be. I don’t mind talking to the nurse; I don’t mind some waiting, but I know that certain situations warrant major attention.

You should have a thorough discussion with your primary care provider when you are well about her approach to dealing with patients with asplenia. You should have a plan to follow in case of illness and fever. For additional information about testing and intervention for asplenia, you and your healthcare provider can refer to the COG’s survivorship guidelines at www.survivorshipguidelines.org .

Because fevers can develop any time, such as when you are on vacation or traveling for business, make a plan for dealing with illness when away from home. One way to do this is to carry a wallet card with pertinent information about your history and risk and/or wear a medical alert emblem. An example of a wallet card for asplenic patients is shown in the information resource (“Health Link”) called “Splenic Precautions” at www.survivorshipguidelines.org .

The following are additional suggestions for survivors with asplenia:

  • Get the pneumococcal vaccine (prevents some types of pneumonia)

  • Get the Hib vaccine (to prevent Hemophilus influenza B)

  • Get the meningococcal vaccine (to prevent meningitis)

  • Get the annual flu vaccine

  • Make sure your healthcare providers know you don’t have a functioning spleen before you have dental work or an invasive procedure such as a colonoscopy

Chapter 16. Immune SystemLymph nodes

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