Aggressive NK-cell leukemia

Name
Aggressive NK-cell leukemia
ICD-O-3 Morphology
9948/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

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Grade
8 - NK (natural killer) cell
Module Rule
None
Alternate Names
Aggresive NK-cell leukemia/lymphoma
Large granular lymphocyte leukemia, NK-cell type-REAL
Definition
Aggressive NK-cell leukemia is characterized by a systemic proliferation of NK cells. This is a disease with an aggressive systemic proliferation of natural killer cells (NK cells) and a rapidly declining 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.
Abstractor Notes
The most common sites of involvement are peripheral blood, bone marrow, liver and spleen; however, dissemination to any organ is possible

Overlap with extranodal NK/T-cell lymphoma showing multi-organ involvement may be possible, but it is unclear whether Aggressive NK-cell leukemia represents the leukemic counterpart of extranodal NK/T-cell lymphoma.

Response to chemotherapy is usually poor.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
1p-
1q+
7p-
11q deletion
del(6)(q21q25)
EBV in a clonal episomal form
Immunophenotyping
CD2+
CD3-
CD3 epsilon+
CD11b+
CD16+
CD56+
CD57-
FAS ligand+
Treatments
Chemotherapy
Radiation
Stem cell transplant
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
207.8 Other specified leukemia
Corresponding ICD-10 Codes
C94.7 Other specified leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C94.8 Other specified leukemias
Signs and Symptoms
Anemia
Coagulopathy
Elevated serum lactate dehydrogenase level
Fever
Hepatosplenogemaly
Hemophagocytic syndrome
Multi-organ failure
Recurrence and Metastases
Relapse is almost the rule in patients achieving remission with or without stem cell transplant.
Epidemiology and Mortality
Age: median age 42
Incidence: rare
Sex: slight male dominance
Survival: 2 month median survival