Aggressive NK-cell leukemia: Difference between revisions
No edit summary |
Category |
||
| Line 126: | Line 126: | ||
[[Category: Oncology]] | [[Category: Oncology]] | ||
[[Category:Up-To-Date]] | |||
[[Category:Oncology]] | |||
[[Category:Medicine]] | |||
[[Category:Hematology]] | |||
[[Category:Immunology]] | |||
Revision as of 01:09, 24 October 2017
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]
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:
- Epstein-Barr virus (EBV) infection
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:
- 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.[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] 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
- ↑ 3.0 3.1 3.2 3.3 3.4 [3] Chan JK. "Natural killer cell neoplasms." Anat Pathol. 1998;3:77-145. PMID 10389582
- ↑ 4.0 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