NK cell leukemia (ANKL): Symptoms, causes, and treatment
Symptoms
Causes and risk factors
Diagnosis
Summary
ANKL is a rare and aggressive type of blood cancer affecting a type of white blood cell called natural killer (NK) cells. In ANKL, the NK cells are abnormal and multiply out of control, crowding out other healthy blood cells. This leads to serious and life threatening complications.
Aggressive natural killer (ANK) cells belong to a group of white blood cells called 'lymphocytes' which play a vital role in the body's immune system. NK cells in particular target and destroy disease-causing pathogens, and detect and control the early signs of cancer.
In ANKL, the NK cells are unable to perform their usual functions. The accumulation of cancerous or 'neoplastic' NK cells in the body leads to serious and life threatening complications.ANKL can present differently from person to person. However, the most common signs and symptoms include: fever
night sweats
unintentional weight loss
swelling of the lymph nodes
People with ANKL commonly present with a rare and life threatening inflammatory condition called 'fulminant hemophagocytic lymphohistiocytosis (HLH). This condition occurs as a result of immune system overactivation, and can lead to multiple organ failure. Common HLH triggers include: infection, with Epstein-Barr virus (EBV), which is the most common cause
acute illness
cancer
The two most common clinical features of HLH are fever and enlargement of the spleen (splenomegaly). The latter may cause symptoms such as: abdominal discomfort
pain in the upper left abdomen
referred pain in the upper left shoulder
abdominal bloating
feeling full after eating very little (early satiety)
weight loss
Due to the rarity of NKL, scientists are still working to determine its causes and risk factors.
While anyone can develop ANKL, the average age at disease onset is 40 years, and the disease is more common among people of Asian ethnicity.
EBV infection is a risk factor for ANKL. As the Centers for Disease Control and Prevention (CDC) explains, EBV is among the most common human viruses in the world, and most people develop EBV at some point in their lifetime. Children rarely experience symptoms, while teenagers and adults may experience symptoms for several weeks or months.
Once a person has had EBV, the virus becomes inactive or 'latent' in their body. However, the virus can express viral proteins that disrupt cell functioning, suppress cell death, and trigger uncontrolled cell growth. This, in turn, can increase the risk of certain cancers.
Cases of ANKL that are associated with EBV infection are referred to as 'EBV-positive'.
ANKL can also develop in people who have never had EBV. Doctors refer to this type of ANKL as 'EBV-negative'. Unlike EBV-positive ANKL, EBV-negative ANKL occurs most often in older adults and arises equally among people of Asian and non-Asian ethnicity.
However, EBV-positive and EBV-negative ANKL appear equally aggressive in terms of their clinical onset and disease course.
Doctors may have difficulty accurately diagnosing ANKL due to the following: The disease is rare and has a rapid onset.
The disease has no specific pathologic features and shares symptoms with many other conditions.
The disease has no standard immunophenotypic features, meaning that the diseased cells do not show any features specific to ANKL, such as specific antigens or markers on their surface.
ANKL can be particularly difficult to diagnose in its early stages due to the small number of cancerous NK cells in the bone marrow. According to some studies, this number may be as low as 5% or even less.
As the disease progresses, the number of cancerous NK cells in the bone marrow rapidly increases. As such, the reviewers recommend that doctors conduct multiple blood or bone marrow specimens at various time points to check for the disease.
Once doctors have retrieved a blood or bone marrow sample, they will send it to a lab for analysis using flow cytometry. This analysis helps determine the types of cells, their characteristics, and the presence of tumor markers. » Learn more:What tests can diagnose leukemia?
Treatment for ANKL typically involves chemotherapy, radiation therapy, or a combination of the two. Large-scale clinical studies are necessary to determine the optimal treatment approach. A person with ANKL can consider talking with their doctor about the possibility of joining clinical trials.
Combined radiation therapy and chemotherapy regimens incorporating the chemotherapy drug 'L-asparaginase' appear most effective for treating ANKL. An example of such a regimen is the 'SMILE' regimen, which consists of the following drugs: dexamethasone
methotrexate
ifosfamide
L-asparaginase
etoposide
» Learn more about leukemia treatment.
The outlook for ANKL is typically very poor , even among individuals on the more successful treatment regimens, such as SMILE.
In a small, older study from 2016, only 50% of 13 individuals on the SMILE regimen experienced a partial or complete response to treatment, with only 27.8% experiencing a complete response to treatment. Eight individuals then received a follow-up blood and bone marrow transplant called 'hematopoietic stem cell transplantation (HSCT)', which involves implanting stem cells to promote healthy blood cell production. Six individuals remained alive following the procedure.
A 2017 study of 21 people with ANKL also demonstrated improved survival rates among those who received HSCT following their initial treatment. Of the 17 individuals who received an L-asparaginase-containing regimen, 14 experienced a complete treatment response prior to HSCT.
At 25-month follow-up, the 2-year progression-free survival (PFS) was 20%, and the overall survival (OS) was 24%. The term 'PFS' refers to the length of time a person lives with a disease without it worsening, while the term 'OS' refers to the average length of time a person lives following their initial diagnosis.
Aggressive natural killer cell leukemia (ANKL) is a rare and aggressive type of blood cancer that affects white blood cells called 'natural killer (NK) cells'. In ANKL, the NK cells are abnormal and multiply out of control, crowding out other healthy blood cells in the blood and bone marrow. This leads to serious and life threatening complications.
The Epstein-Barr virus (EBV) is a risk factor for ANKL. Cases of NKL related to EBV are referred to as 'EBV-positive', while those unrelated to EBV are referred to as 'EBV-negative'.
Due to the rarity and aggressiveness of ANKL, doctors have yet to establish a standard treatment approach. However, treatments incorporating the chemotherapy drug L-asparaginase appear to be the most effective.
The current outlook for ANKL is poor. Research is ongoing to help determine more effective treatments.
Leukemia
Blood / Hematology
Cancer / Oncology Medical News Today has strict sourcing guidelines and relies on peer-reviewed studies, academic research institutions, and medical journals and associations. We only use quality, credible sources to ensure content accuracy and integrity. You can learn more about how we ensure our content is accurate and current by reading our editorial policy. About Epstein-Barr virus. (2024).
https://www.cdc.gov/epstein-barr/about/index.html
Chapman J, et al. (2023). Splenomegaly.
https://www.ncbi.nlm.nih.gov/books/NBK430907/
Eissmann, P. (n.d.). Natural killer cells.
https://www.immunology.org/public-information/bitesized-immunology/cells/natural-killer-cells
El Hussein S, et al. (2020). Aggressive NK cell leukemia: Current state of the art.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7600035/
Hamadani M, et al. (2017). Allogeneic hematopoietic cell transplantation for aggressive NK cell leukemia. A center for international blood and marrow transplant research analysis.
https://www.sciencedirect.com/science/article/pii/S1083879117302379
Hines M, et al. (2018). Diagnosis, treatment, and management of hemophagocytic lymphohistiocytosis in the critical care unit. Critical care of the pediatric immunocompromised hematology/oncology patient.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7123852/
Jung K. S, et al. (2016). L-asparaginase-based regimens followed by allogeneic hematopoietic stem cell transplantation improve outcomes in aggressive natural killer cell leukemia.
https://link.springer.com/content/pdf/10.1186/s13045-016-0271-4.pdf
Shannon-Lowe C, et al. (2017). Epstein-Barr virus-associated lymphomas.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5597738/#:~:text=Epstein%E2%80%93Barr%20virus%20(EBV),issue%20'Human%20oncogenic%20viruses'.
Spaner C, et al. (2024). Case report: Aggressive natural killer cell leukemia and refractory hemophagocytic lymphohistiocytosis in an adolescent.
https://www.frontiersin.org/journals/hematology/articles/10.3389/frhem.2024.1413794/full
Sumbly V, et al. (2022). Aggressive natural killer cell leukemia: A brief overview of its genomic landscape, histological features, and current management.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8956279/
Tse E, et al. (2017). The diagnosis and management of NK/T-cell lymphomas.
https://jhoonline.biomedcentral.com/articles/10.1186/s13045-017-0452-9
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