Aggressive NK-cell leukemia: Difference between revisions
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*There is no classification system for aggressive NK-cell leukemia. | *There is no classification system for aggressive NK-cell leukemia. | ||
==Pathophysiology== | ==Pathophysiology== | ||
*The pathogenesis of aggressive NK-cell leukemia is characterized by | *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. | *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: | *On gross pathology, characteristic findings of aggressive NK-cell leukemia, include: | ||
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:*CD11b and CD16 show variable expression | :*CD11b and CD16 show variable expression | ||
:*The table below demonstrates the immunophenotype for patients with aggressive NK-cell leukemia. | :*The table below demonstrates the immunophenotype for patients with aggressive NK-cell leukemia. | ||
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| rowspan="1"| '''Status''' | | rowspan="1"| '''Status''' | ||
Revision as of 17:22, 5 May 2016
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.
Classification
- There is no classification system for aggressive NK-cell leukemia.
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:
- 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.
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
- [Differential dx2]
- [Differential dx3]
Epidemiology and Demographics
- Aggressive NK-cell leukemia is very uncommon.
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:
- Epstein-Barr virus (EBV) infection
Natural History, Complications and Prognosis
- The majority of patients with aggressive NK-cell leukemia are symptomatic at diagnosis.
- Early clinical features include fatigue, night sweats, and fever.
- If left untreated, patients with aggressive NK-cell leukemia may progress to develop
- Common complications of aggressive NK-cell leukemia, include:
- Coagulopathies
- Hemophagocytic syndrome
- Multiple organ failure
- Myelofibrosis
- Prognosis is generally poor, and the median survival rate of patients with aggressive NK-cell leukemia is approximately 12 months
Diagnosis
Symptoms
- Aggressive NK-cell leukemia is usually asymptomatic.
- Symptoms of aggressive NK-cell leukemia may include the following:
- 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:
- Hepatoesplenomegaly
Laboratory Findings
- Laboratory findings consistent with the diagnosis of aggressive NK-cell leukemia, include:
- 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:
- 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.
- Other novel treatments may include pralatrexate.
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] 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] 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] Chan JK. "Natural killer cell neoplasms." Anat Pathol. 1998;3:77-145. PMID 10389582