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.


Overview

Historical Perspective

  • [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
  • In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • [Disease name] may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

  • The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Clinical Features

Differentiating [disease name] from other Diseases

  • [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Patients of all age groups may develop [disease name].
  • [Disease name] is more commonly observed among patients aged [age range] years old.
  • [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

  • [Disease name] affects men and women equally.
  • [Gender 1] are more commonly affected with [disease name] than [gender 2].
  • The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

  • There is no racial predilection for [disease name].
  • [Disease name] usually affects individuals of the [race 1] race.
  • [Race 2] individuals are less likely to develop [disease name].

Risk Factors

  • Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

  • The majority of patients with [disease name] remain asymptomatic for [duration/years].
  • Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

  • The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • [Disease name] is usually asymptomatic.
  • Symptoms of [disease name] may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]
  • [symptom 5]
  • [symptom 6]

Physical Examination

  • Patients with [disease name] usually appear [general appearance].
  • Physical examination may be remarkable for:
  • [finding 1]
  • [finding 2]
  • [finding 3]
  • [finding 4]
  • [finding 5]
  • [finding 6]

Laboratory Findings

  • There are no specific laboratory findings associated with [disease name].
  • A [positive/negative] [test name] is diagnostic of [disease name].
  • An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
  • Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

  • There are no [imaging study] findings associated with [disease name].
  • [Imaging study 1] is the imaging modality of choice for [disease name].
  • On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
  • [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

  • [Disease name] may also be diagnosed using [diagnostic study name].
  • Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

  • There is no treatment for [disease name]; the mainstay of therapy is supportive care.
  • The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action 1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for [disease name].
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
  • [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

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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