Name

ICD-O-3 Morphology

9948/3: Aggressive NK-cell leukemia
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

Module Rule

None

Alternate Names

Aggresive NK-cell leukemia/lymphoma

Definition

Aggressive NK-cell leukemia is a systemic neoplastic proliferation of NK cells frequently associated with EBV.

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

del(6)(q21q25)
EBV positive

Immunophenotyping

CD2 positive
CD3 negative
CD3 epsilon positive
CD5 negative
CD11b expression
CD16 positive
CD56 positive
CD57 negative
FASL expression

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Radiation therapy

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 (U.S. only)

C94.8 Other specified leukemias (effective October 01, 2015)

Signs and Symptoms

Coagulopathy
Elevated serum lactate dehydrogenase level
Hepatosplenogemaly
Hemophagocytic syndrome
Multi-organ failure

Progression and Transformation

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

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 353-354

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2
Glossary