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Large granular lymphocyte leukemia, NK-cell type-REAL
Aggressive NK-cell leukemia is characterized by a systemic proliferation of NK cells. The disease has an aggressive clinical course. Since the number of neoplastic cells in the peripheral blood and marrow can be limited, the disease is different from the usual leukemias and thus has been called aggressive NK-cell leukemia/lymphoma in the past.
This is a disease with an aggressive systemic proiferation of natural killer cells (NK cells) and a rapidly declining clinical course.
The most common sites of involvement are peripheral blood, bone marrow, liver and spleen; but this neoplasm can disseminate to any organ. Since the number of neoplastic cells in the peripheral blood and bone marrow can be limited, the disease is different from the usual leukemia and that is why it is called Aggressive NK-cell leukemia/lymphoma. There can be overlap with extranodal NK/T-cell lymphoma showing multi-organ involvement, but it is unclear whether Aggressive NK-cell leukemia represents the leukemic counterpart of extranodal NK/T-cell lymphoma. Patients usually present with fever, constitutional symptoms and a leukemic blood picture. The disease course is frequently complicated by multi-organ failure, coagulopathy and hemophagocytic syndrome. The median survival is less than 2 months. Response to chemotherapy is usually poor and relapse is almost the rule in patients achieving remission with or without stem cell transplant.