Aggressive NK-cell leukemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [3]
Synonyms and keywords: ANKL

Overview

Aggressive NK-cell leukemia is a disease with an aggressive, systemic proliferation of natural killer cells (NK cells) and a rapidly declining clinical course. There is no classification system for aggressive NK-cell leukemia. The pathogenesis of aggressive NK-cell leukemia is characterized by the proliferation of natural killer cells. Aggressive NK-cell leukemia is very uncommon, and is most commonly seen among middle aged adults. The most common risk factors in the development of aggressive NK-cell leukemia is the Epstein-Barr virus (EBV) infection. The majority of patients with aggressive NK-cell leukemia are symptomatic at diagnosis. Early clinical features include fatigue, night sweats, and fever. Physical examination among patients with aggressive NK-cell leukemia may be remarkable for hepatosplenomegaly. Aggressive NK-cell leukemia may also be diagnosed using bone marrow biopsy, findings of which can include extensive marrow replacement by leukemic cells and reactive histiocytes displaying hemophagocytosis. The mainstay of therapy for aggressive NK-cell leukemia is anthracycline-containing chemotherapy.

Historical Perspective

  • In 1986, Dr. Fernandez and his colleagues reported the first case of aggressive NK-cell leukemia.[1]

Classification

Pathophysiology

  • No remarkable findings
Status Antigens
Positive CD2, CD3ε, CD56, perforin, granzyme B, TIA-1, CCR5
Negative CD57

Causes

There are no established causes for aggressive NK-cell leukemia.

Differentiating Aggressive NK-cell Leukemia from Other Diseases

Aggressive NK-cell leukemia must be differentiated from other diseases that cause fever, fatigue, and lymphadenopathy such as:

Epidemiology and Demographics

  • Aggressive NK-cell leukemia is a rare disorder.[4]
  • Aggressive NK-cell leukemia is more commonly observed among young adults with median age of 42 years old.
  • Aggressive NK-cell leukemia affects men more frequently than women.
  • Aggressive NK-cell leukemia usually affects individuals of the Asian race.

Risk Factors

Common risk factors in the development of aggressive NK-cell leukemia include Epstein-Barr virus (EBV) infection[4]

Screening

  • There is insufficient evidence to recommend routine screening for aggressive NK-cell leukemia.

Natural History, Complications, and Prognosis

Diagnosis

Symptoms

  • Symptoms of aggressive NK-cell leukemia may include the following:[5]

Physical Examination

  • Patients with aggressive NK-cell leukemia usually appear icteric and malnourished.[6]
  • Physical examination may be remarkable for:

Laboratory Findings

  • Laboratory findings consistent with the diagnosis of aggressive NK-cell leukemia include:

Peripheral Blood Smear

  • Large granular lymphocyte (LGL)
  • Fine or coarse azurophilic granules and nucleoli of varying prominence
  • Nuclei may be irregular and hyperchromatic

Imaging Findings

There are no imaging findings associated with aggressive NK-cell leukemia.

Other Diagnostic Studies

  • Aggressive NK-cell leukemia may also be diagnosed using bone marrow biopsy.
  • Findings on bone marrow biopsy, include:
  • Extensive marrow replacement by leukemic cells
  • Reactive histiocytes displaying hemophagocytosis

Treatment

Medical Therapy

  • The mainstay of therapy for aggressive NK-cell leukemia is anthracycline-containing chemotherapy.
  • L-asparaginase containing chemotherapy regimen followed by allogeneic stem cell transplantation shows to slightly prolong life, but relapse is almost assured.[7]
  • Other novel treatments may include pralatrexate.

Surgery

  • Surgery is not recommended among patients with aggressive NK-cell leukemia.

Primary Prevention =

  • There are no primary preventive measures available for aggressive NK-cell leukemia.

Secondary Prevention =

  • There are no secondary preventive measures available for aggressive NK-cell leukemia.

References

  1. Fernandez LA, Pope B, Lee C, Zayed E (April 1986). "Aggressive natural killer cell leukemia in an adult with establishment of an NK cell line". Blood. 67 (4): 925–30. PMID 3955237.
  2. Ishida, Fumihiro (2018). "Aggressive NK-Cell Leukemia". Frontiers in Pediatrics. 6. doi:10.3389/fped.2018.00292. ISSN 2296-2360.
  3. 3.0 3.1 [1] Chan JK. "Natural killer cell neoplasms." Anat Pathol. 1998;3:77-145. PMID 10389582
  4. 4.0 4.1 Suzuki, R; Suzumiya, J; Nakamura, S; Aoki, S; Notoya, A; Ozaki, S; Gondo, H; Hino, N; Mori, H; Sugimori, H; Kawa, K; Oshimi, K (2004). "Aggressive natural killer-cell leukemia revisited: large granular lymphocyte leukemia of cytotoxic NK cells". Leukemia. 18 (4): 763–770. doi:10.1038/sj.leu.2403262. ISSN 0887-6924.
  5. Song SY, Kim WS, Ko YH, Kim K, Lee MH, Park K (December 2002). "Aggressive natural killer cell leukemia: clinical features and treatment outcome". Haematologica. 87 (12): 1343–5. PMID 12495907.
  6. Li, Chunrui; Tian, Ye; Wang, Jue; Zhu, Li; Huang, Liang; Wang, Na; Xu, Danmei; Cao, Yang; Li, Jianyong; Zhou, Jianfeng (2014). "Abnormal immunophenotype provides a key diagnostic marker: a report of 29 cases of de novo aggressive natural killer cell leukemia". Translational Research. 163 (6): 565–577. doi:10.1016/j.trsl.2014.01.010. ISSN 1931-5244.
  7. Ishida F, Ko YH, Kim WS, Suzumiya J, Isobe Y, Oshimi K, Nakamura S, Suzuki R (June 2012). "Aggressive natural killer cell leukemia: therapeutic potential of L-asparaginase and allogeneic hematopoietic stem cell transplantation". Cancer Sci. 103 (6): 1079–83. doi:10.1111/j.1349-7006.2012.02251.x. PMID 22360679.