Abstract
Aggressive natural killer-cell leukemia (ANKL) is a rare hematological malignancy characterized by the abnormal proliferation of natural killer (NK) cells. There are currently no therapies approved by the US Food and Drug Administration (FDA) for the treatment of ANKL, but advancements in genomics are assisting in the unraveling of this rare malignancy. We selected 37 articles that contained information on genomics, immunohistochemistry, and/or current clinical trials relating to the treatment and survival of ANKL. Current therapeutic strategies have been subdivided into (1) concurrent chemoradiation, (2) sequential chemoradiation, and (3) sandwich chemoradiation. These methods have been developed to reduce toxicity while still producing a pathologic response. Concurrent chemoradiation with VIDL (etoposide, ifosfamide, dexamethasone, and L-asparaginase) produced an excellent clinical response, while sequential chemoradiation with SMILE (steroid dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide) showed an adequate response, but with severe hematologic toxicity. The efficacy of L-asparaginase in chemotherapeutic regimens and its association with NK-cell apoptosis have led to its inclusion in all standard regimens. Future studies are focusing on the addition of a programmed death-ligand 1 (PD-L1) inhibitor and hematopoietic stem cell transplant (HSCT).
Highlights
Aggressive natural killer-cell leukemia (ANKL) is a rare hematological malignancy characterized by the abnormal proliferation of natural killer (NK) cells
There are currently no therapies approved by the US Food and Drug Administration (FDA) for the treatment of ANKL, but advancements in genomics are assisting in the unraveling of this rare malignancy
In the 2016 World Health Organization (WHO) classification, ANKL is classified as a type of "Mature T and NK neoplasm" and has an association with the Epstein-Barr Virus (EBV) [1]
Summary
Aggressive natural killer-cell leukemia (ANKL) is a rare hematological malignancy characterized by the abnormal proliferation of natural killer (NK) cells. Males are twice as likely as females to be diagnosed with ANKL, with an age distribution that favors older populations, except for young adult Asian males [2,3,4,5]. There are currently no therapies approved by the US Food and Drug Administration (FDA) for the treatment of ANKL, advancements in the field of genomics have considerably assisted scientists in better understanding this malignancy. We present a brief overview of ANKL with consideration of its genomic landscape and histologic features, with a discussion of the current treatment regimens and the challenges experienced in the treatment of this aggressive, deadly disease
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