Aggressive NK-cell leukemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [4] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [5]

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.[1][2][3] 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 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.[4]

Classification

  • There is no classification system for aggressive NK-cell leukemia.[3]

Pathophysiology

  • The pathogenesis of aggressive NK-cell leukemia is characterized by the aggressive, systemic proliferation of natural killer cells.
  • The mutation in oncogene p53 has been associated with the development of aggressive NK-cell leukemia.
  • On gross pathology, characteristic findings of aggressive NK-cell leukemia, include:
  • No remarkable findings
  • On microscopic histopathological analysis, characteristic findings of aggressive NK-cell leukemia, include:[3]
  • Large cells with abundant blue cytoplasm
  • Azurophilic granules
  • Irregular nuclei
  • Open chromatin
  • Distinct nucleoli
  • CD11b and CD16 show variable expression
  • The table below demonstrates the immunophenotype for patients with aggressive NK-cell leukemia.[3]
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:
  • Human immunodeficiency virus
  • Chronic neutrophilic leukemia
  • Juvenile myelomonocytic leukemia

Epidemiology and Demographics

  • Aggressive NK-cell leukemia is very uncommon.[4]

Age

  • Aggressive NK-cell leukemia is more commonly observed among middle aged adults.

Gender

  • Aggressive NK-cell leukemia affects men and women equally.

Race

  • Aggressive NK-cell leukemia usually affects individuals of the Asians race.

Risk Factors

  • Common risk factors in the development of aggressive NK-cell leukemia, include:

Natural History, Complications and Prognosis

  • The majority of patients with aggressive NK-cell leukemia are symptomatic at diagnosis.[4]
  • Early clinical features include fatigue, night sweats, and fever.
  • If left untreated, patients with aggressive NK-cell leukemia may progress to develop recurrent infections.
  • Common complications of aggressive NK-cell leukemia, include:
  • Prognosis is generally poor, and the median survival rate of patients with aggressive NK-cell leukemia is approximately 12 months.[4]

Diagnosis

Symptoms

  • Aggressive NK-cell leukemia is usually asymptomatic.
  • Symptoms of aggressive NK-cell leukemia may include the following:[3]
  • Fever
  • Swelling in the lymph nodes in the neck
  • Night sweats
  • Persistent fatigue
  • Loss of appetite
  • Nausea
  • Vomiting

Physical Examination

  • Patients with aggressive NK-cell leukemia usually appear pale and malnourished.
  • Physical examination may be remarkable for:[4]
  • Hepatoesplenomegaly

Laboratory Findings

  • Laboratory findings consistent with the diagnosis of aggressive NK-cell leukemia, include:[4]

Peripheral Blood Smear

  • Large granular lymphocyte (LGL)
  • 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:[4]
  • 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.[4]
  • Other novel treatments may include pralatrexate.[4]

Surgery

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

Prevention

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

References

  1. [1] Chan JK, Sin VC, Wong KF, Ng CS, Tsang WY, Chan CH, Cheung MM, Lau WH. "Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm." Blood. 1997 Jun 15;89(12):4501-13. PMID 9192774
  2. [2] Imamura N, Kusunoki Y, Kawa-Ha K, Yumura K, Hara J, Oda K, Abe K, Dohy H, Inada T, Kajihara H, et al. "Aggressive natural killer cell leukaemia/lymphoma: report of four cases and review of the literature. Possible existence of a new clinical entity originating from the third lineage of lymphoid cells." Br J Haematol. 1990 May;75(1):49-59. PMID 2375924
  3. 3.0 3.1 3.2 3.3 3.4 [3] Chan JK. "Natural killer cell neoplasms." Anat Pathol. 1998;3:77-145. PMID 10389582
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 Aggressive NK-cell leukemia. https://en.wikipedia.org/wiki/Aggressive_NK-cell_leukemia Accessed on May 5, 2016

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