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Aggressive NK-cell leukemia

Natural killer (NK) cells are a type of lymphocyte (called large granular lymphocytes) that attacks foreign cells. They are often the body’s first
line of defence against infection.

Aggressive NK-cell leukemia (also called aggressive NK-cell lymphoma, or ANKL, is a very rare type of NHL. The body makes large numbers
of NK cells that are larger than normal. It is grouped with T-cell lymphomas.

ANKL develops most often in people from Asia, Central America and South America. Doctors don’t know the exact cause of ANKL, but it has a
strong link with the Epstein-Barr virus (EBV). ANKL usually progresses quickly and often has a poor prognosis.

There is a very rare slow-growing (indolent) type of NK-cell leukemia that has a more favourable prognosis. It is called chronic NK-cell
leukemia and is treated like T-cell large granular lymphocytic leukemia.

Symptoms
ANKL usually affects the blood and bone marrow, but it can also affect the liver or spleen. People with ANKL usually have:

 B symptoms (unexplained fever, drenching night sweats and unexplained weight loss) that develop quickly
 larger than normal liver or spleen
 lower than normal numbers of healthy blood cells (called cytopenia)
 larger than normal lymph nodes
In some cases, ANKL can cause disseminated intravascular coagulation (DIC). Different organs can also stop working, or fail. These are serious
conditions that need to be treated right away.

Treatments
ANKL is treated with chemotherapy. Some people may be offered a stem cell transplant.

Chemotherapy
Chemotherapy is the main treatment for ANKL.

A combination of the following chemotherapy drugs is usually given for ANKL:

 vincristine (Oncovin)
 daunorubicin (Cerubidine, daunomycin)
 doxorubicin (Adriamycin)
 cytarabine (Cytosar, Ara-C)
 methotrexate
 cyclophosphamide (Cytoxan, Procytox)
Steroids, such as prednisone or dexamethasone (Decadron, Dexasone), may be used in combination with chemotherapy.

Central nervous system prophylaxis or treatment


The central nervous system (CNS) is the brain and spinal cord. There is a risk that ANKL will spread to the CNS. CNS prophylaxis is used to try
to prevent lymphoma cells from entering the tissue covering the brain and spinal cord.

CNS prophylaxis or treatment may be given as intrathecal chemotherapy. This means that the chemotherapy drugs are injected into the
cerebrospinal fluid (CSF).

The most common chemotherapy drugs used for CNS prophylaxis or treatment are:

 methotrexate
 cytarabine (Cytosar, Ara-C)
 a steroid such as prednisone

 Signs and symptoms[edit]


 Patients usually present with constitutional symptoms (malaise, weight loss, fatigue),
and hepatosplenomegaly is commonly found on physical exam. Lymphadenopathy is also found to a lesser
extent. Due to the aggressive nature of the disease, patients may initially present at a more advanced stage,
with coagulopathies, hemophagocytic syndrome, and multi-organ failure.[1][2][5][6][7] Rarely, individuals who have
an aggressive NK cell lymphoma that is associated with latent infection with the Epstein-Barr virus (see next
section) present with or develop extensive allergic reactions to mosquito bites. The symptoms of these
reactions range from a greatly enlarged bite site that may be painful and involve necrosis to systemic
symptoms (e.g. fever, swollen lymph nodes, abdominal pain, and diarrhea), or, in extremely rare cases, to
life-threatening anaphylaxis.[8]

 Cause[edit]
 This disease has a strong association with the Epstein-Barr virus (EBV),[9] but the true pathogenesis of this
disease has yet to be described. The cell of origin is believed to be an NK cell.[4] Blastoid NK cell lymphoma
appears to be a different entity and shows no association with EBV. [1]
 Sites of involvement[edit]
 This disease is typically found and diagnosed in peripheral blood, and while it can involve any organ, it is
usually found in the spleen, liver, and bone marrow.[4]

 Diagnosis[edit]
 Leukemic cells are invariably present in samples of peripheral blood to a variable
extent. Pancytopenia (anemia, neutropenia, thrombocytopenia) is commonly seen as well.[4]
 Peripheral blood[edit]
 The leukemic cells have a diameter mildly greater than a large granular lymphocyte (LGL) and have
azurophilic granules and nucleoli of varying prominence. Nuclei may be irregular and hyperchromatic. [4]
 Bone marrow[edit]
 Bone marrow involvement runs the spectrum between an inconspicuous infiltrate to extensive marrow
replacement by leukemic cells. Reactive histiocytes displaying hemophagocytosis can be seen interspersed
in the neoplastic infiltrate.[4]
 Other organs[edit]
 Leukemic involvement of organs is typically destructive on tissue sections with necrosis and possibly
angioinvasion, and the monotonous infiltrate may be diffuse or patchy. [4]
 Immunophenotype[edit]
 The immunophenotype of this disease is the same as extranodal NK/T-cell lymphoma, nasal type and is
shown in the table below. CD11b and CD16 show variable expression.[1][10]

Status Antigens

Positive CD2, CD3ε, CD56, perforin, granzyme B, TIA-1, CCR5

Negative CD57

 Genetic findings[edit]
 Due to the NK lineage, clonal rearrangements of lymphoid (T cell receptor; B cell receptor) genes are not
seen.[4] The genome of the Epstein Barr virus (EBV) is detected in many cases,[9] along with a variety of
chromosomal abnormalities.[11]

 Treatment[edit]
 Currently Aggressive NK-cell leukemia, being a subtype of PTCL, is treated similarly to B-cell lymphomas.
However, in recent years, scientists have developed techniques to better recognize the different types of
lymphomas, such as PTCL. It is now understood that PTCL behaves differently from B-cell lymphomas and
therapies are being developed that specifically target these types of lymphoma. Currently, however, there
are no therapies approved by the U.S. Food and Drug Administration (FDA) specifically for
PTCL. Anthracycline-containing chemotherapy regimens are commonly offered as the initial therapy. Some
patients may receive a stem cell transplant.[12][13][14][15][16] Novel approaches to the treatment of PTCL in the
relapsed or refractory setting are under investigation.

Risk factors
Factors that may increase your risk of developing some types of leukemia include:

 Previous cancer treatment. People who've had certain types of chemotherapy and radiation
therapy for other cancers have an increased risk of developing certain types of leukemia.

 Genetic disorders. Genetic abnormalities seem to play a role in the development of leukemia.
Certain genetic disorders, such as Down syndrome, are associated with an increased risk of
leukemia.

 Exposure to certain chemicals. Exposure to certain chemicals, such as benzene — which is found
in gasoline and is used by the chemical industry — is linked to an increased risk of some kinds of
leukemia.

 Smoking. Smoking cigarettes increases the risk of acute myelogenous leukemia.


 Family history of leukemia. If members of your family have been diagnosed with leukemia, your
risk of the disease may be increased.

However, most people with known risk factors don't get leukemia. And many people with leukemia have
none of these risk factors.

Reducing your risk for leukemia


You may lower your risk of developing leukemia by doing the following.

Be a non-smoker
Not smoking is the best way to lower your risk of leukemia. Don’t start smoking. If you smoke, get help to quit.
Quitting smoking lowers your risk of leukemia.

Maintain a healthy body weight


Some studies have shown that overweight and obesity may increase your risk of leukemia. You may lower your
risk by having a healthy body weight. Eating well and being physically active can help you have a healthy body
weight.

Avoid breathing in benzene and formaldehyde


Avoid or lower your long-term contact with benzene and formaldehyde to reduce your risk of leukemia.

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