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Autoimmune hemolytic anemia (AIHA) is a rare blood disorder that causes the immune system to attack and destroy your red blood cells. If you’re living with AIHA, you may wonder what caused it or if any other health conditions are related to it.
While primary AIHA has no known underlying cause, secondary AIHA usually arises due to another illness or virus. Blood cancers like lymphoma have been found to cause secondary AIHA in some people.
Here, we’ll discuss the connection between AIHA, lymphoma, and other types of blood cancer. We’ll also examine the relationship between AIHA and anticancer medications, exploring treatment options for people living with AIHA and blood cancer.
Although AIHA is an autoimmune disease that may have no known cause, it’s sometimes caused by underlying blood cancer. In this case, AIHA is seen as a complication of blood cancer, or a condition that occurs during or after an existing disease or its treatment.
One member of myAIHAteam, the online social support group for people with AIHA, wrote that their spouse’s AIHA was caused by an “aggressive lymphoma.”
AIHA is associated with several different types of blood cancers and disorders, including:
Chronic lymphocytic leukemia (CLL) is the most common type of leukemia among adults. This type of cancer begins in the white blood cells (lymphocytes) and can travel into the blood. People with CLL may not show symptoms in the first couple of years, as it can take time for leukemia cells to grow.
According to one literature review, AIHA is a complication in 7 percent to 10 percent of CLL cases. Researchers observed that the connection between AIHA and CLL was underlined by complex processes of the immune system. They also noted the possibility that AIHA could be caused by CLL therapies like fludarabine (a chemotherapy drug).
The diagnosis of AIHA caused by CLL follows a process similar to diagnosing primary AIHA.
AIHA caused by CLL requires the following criteria for diagnosis:
If your AIHA is caused by CLL, it’s important to treat the underlying CLL. This process goes for all cases of secondary AIHA — treating the underlying cause will help treat the AIHA. Depending on your specific case of CLL, your doctor may use several additional treatment options to also treat your AIHA.
Learn more about AIHA treatments here.
Non-Hodgkin lymphoma (NHL) is a type of blood cancer that occurs when malignant (cancerous) cells develop in the lymphatic system, which is a network of tissues and organs that move the lymph in your bloodstream. Non-Hodgkin lymphoma can be diagnosed at any age and causes symptoms like enlarged lymph nodes, fever, and unexplained weight loss.
About 20 percent of people with AIHA develop lymphoma. In rare instances, non-Hodgkin lymphoma increases the risk of AIHA. When people have non-Hodgkin lymphoma alongside AIHA, it’s called AIHA-associated NHL (AIHA/NHL). There is not a lot of research on the prevalence (occurrence) of AIHA/NHL, but a recent study on 20 people with NHL and AIHA found that 0.91 percent of people with NHL also had AIHA, and 9.8 percent of people with AIHA also had NHL. Researchers found that people with AIHA/NHL were more likely to be older, have more advanced NHL, and have multiple types of NHL.
The risk of AIHA also varies depending on the type of non-Hodgkin lymphoma. Research from the journal Frontiers in Immunology has found that AIHA occurs in:
Waldenstrӧm macroglobulinemia is a rare type of non-Hodgkin lymphoma. One review from the journal Clinical Lymphoma Myeloma and Leukemia identified a personal history of AIHA as a risk factor — a factor that increases the risk of developing WM.
As with CLL, the presence of AIHA with NHL is connected to dysfunction of the immune system that destroys red blood cells. Treatment depends on your specific case but will likely involve a combination of treating the NHL and managing AIHA symptoms.
Multiple myeloma is a blood cancer that starts in plasma cells, a type of white blood cell. This type of blood cancer is more common in African Americans and older people.
Like non-Hodgkin lymphoma, AIHA coexisting with multiple myeloma is rare. One study found that about 10.6 percent of participants with multiple myeloma also had AIHA. One systematic review found the occurrence of AIHA in people with multiple myeloma to vary from 4 percent to 10 percent.
The association between multiple myeloma and AIHA is not well understood, but it’s possibly related to immune system disturbances that allow the immune system to attack its own red blood cells. Research from the journal BBA Clinical indicates that people with AIHA who have multiple myeloma may enter remission (a period of lessened symptoms) after the multiple myeloma is treated.
A related disorder called monoclonal gammopathy of undetermined significance will sometimes turn into myeloma. An article in the American Journal of Hematology cites that people with AIHA are six times more likely to have MGUS compared to people without AIHA. However, researchers aren’t sure whether MGUS or AIHA usually develops first because many people with MGUS don’t know that they have the condition.
Researchers have found connections between anticancer medications and AIHA. Some anticancer drugs increase the risk of AIHA. On the other hand, some AIHA medications are associated with an increased risk of cancer.
In rare instances, people with blood cancer may develop AIHA from taking medications aimed at treating cancer. AIHA can develop in individuals with blood cancer who are undergoing treatment with immune checkpoint inhibitors like nivolumab. An immune checkpoint inhibitor is a medication that helps the immune system fight cancer.
AIHA has been found to develop in people who undergo chimeric antigen receptor (CAR) T-cell therapy, which may be used to treat blood cancers. In CAR T-cell therapy, genetically modified immune cells are infused into a patient to help them fight cancer. AIHA has also been linked to therapy for WM — specifically, the use of cladribine.
Some AIHA medications are associated with an increased risk of cancer. For example, AIHA treatments like azathioprine, cyclosporine, and mycophenolate may be associated with a higher chance of developing certain types of cancer. For example, cyclosporine can increase the risk of lymphoma.
If AIHA is a complication of blood cancer, treating the blood cancer should help treat the AIHA. There are several different treatment options for people living with both conditions.
Rituximab (Rituxan) is a cancer medication administered by intravenous (IV) infusion. The medication activates the immune system to attack and destroy cancer cells. Rituximab is often combined with chemotherapy but can be used alone. Research from the journals The Oncologist and Journal of Clinical Medicine has shown this cancer drug effectively reduces symptoms of CLL and AIHA.
Cyclophosphamide (Cytoxan, Neosar) is an oral medication used to manage and treat blood cancers like multiple myeloma and non-Hodgkin lymphoma. Like rituximab, cyclophosphamide is used in combination with chemotherapy treatment. Low doses of this treatment with corticosteroid medications (like prednisone) have been shown to help stabilize symptoms for people with AIHA.
A splenectomy is a surgical procedure to remove the spleen when it can no longer fight off infections or filter out old and damaged blood cells. Splenectomies may be performed on those with hematologic or blood disorders like AIHA. People with blood cancers may develop leukemia in their spleen, which can affect blood cell counts. In these cases, a splenectomy may also be considered.
If you have AIHA and are concerned about your risk of blood cancer, talk to your hematology provider. If deemed necessary, they will refer you to an oncology specialist for testing and walk you through your options and risk factors.
On myAIHAteam, people with autoimmune hemolytic anemia and their loved ones come together to ask questions, give advice, and share their stories with others who understand life with this rare disease.
Are you living with AIHA and blood cancer? How have you managed both conditions? Share your experience in the comments below, or start a conversation by posting on your Activities page.
Danielle Kurant, M.D.
currently works in research at the Dana-Farber Cancer Institute.
Learn more about her here.
Taneia Surles
is a health and wellness writer and public health professional.
Learn more about her here.
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