Aggressive Granular Lymphocyte Leukemia of Natural Killer Cell Type in an Elderly Patient.

Ichikawa, Naoaki; Kitano, Kiyoshi; Higuchi, Makoto; Kobayashi, Hikaru; Terada, Nobuo; Shimodaira, Shigetaka; Sonoyama, Masayuki; Inoue, Masami; Kawa, Keisei; Furuta, Seiichi

doi:10.2169/internalmedicine.32.882

Cited by 2 publications

(2 citation statements)

References 15 publications

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“…Therefore, ANKL should be recognized as a malignancy among adolescent and young adult (AYA) populations. Approximately 400 cases have been reported, with an increasing number of patients being reported from Caucasian populations and Latin American areas ( 4 – 6 , 12 , 15 – 20 ). Whether or not clinical characteristics differ geographically or with ethnic backgrounds is unclear at present.…”

Section: Epidemiologymentioning

confidence: 99%

Aggressive NK-Cell Leukemia

Ishida

2018

Front. Pediatr.

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Aggressive NK cell leukemia (ANKL) is a rare malignant lymphoproliferative disorder of mature NK cells closely associated with Epstein-Barr virus (EBV) and more common in East Asia than in other areas. Significant variations exist in the morphology of ANKL tumor cells, from typical large granular lymphocyte morphology to highly atypical features with basophilic cytoplasm containing azurophilc granules. The main involved sites are hepatosplenic lesions, bone marrow and peripheral blood, and nasal or skin lesions are infrequent. A fever and liver dysfunction with an often rapidly progressive course are the main clinical symptoms, including hemophagocytic syndrome and disseminated intravascular coagulation. Although the outcome had been dismal for decades, with a median survival of less than three months, the introduction of combined chemotherapy including L-asparaginase and allogeneic hematopoietic cell transplantation has helped achieve a complete response and potential cure for some patients. With the advent of next-generation sequencing technologies, molecular alterations of ANKL have been elucidated, and dysfunctions in several signaling pathways, including the JAK/STAT pathway, have been identified. Novel target approaches to managing these abnormalities might help improve the prognosis of patients with ANKL.

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Section: Epidemiologymentioning

confidence: 99%

Aggressive NK-Cell Leukemia

Ishida

2018

Front. Pediatr.

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“…The three patients of ANKL have been reported elsewhere. [15][16][17] Diagnoses were based on the published criteria. 18,19 Six healthy volunteers were also included as controls.…”

Section: Patients and Healthy Donorsmentioning

confidence: 99%

Significance of chemokine receptor expression in aggressive NK cell leukemia

et al. 2005

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Natural killer (NK) cell-type lymphoproliferative diseases of granular lymphocytes can be subdivided into aggressive NK cell leukemia (ANKL) and chronic NK cell lymphocytosis (CNKL). One reason for the poor outcome in ANKL is leukemic infiltration into multiple organs. The mechanisms of cell trafficking associated with the chemokine system have been investigated in NK cells. To clarify the mechanism of systemic migration of leukemic NK cells, we enrolled nine ANKL and six CNKL cases, and analyzed the expression profiles and functions of chemokine receptors by flowcytometry and chemotaxis assay. CXCR1 was detected on NK cells in all groups, and CCR5 was positive in all ANKL cells. Proliferating NK cells were simultaneously positive for CXCR1 and CCR5 in all ANKL patients examined, and NK cells with this phenotype did not expand in CNKL patients or healthy donors. ANKL cells showed enhanced chemotaxis toward the ligands of these receptors. These results indicated that the chemokine system might play an important role in the pathophysiology of ANKL and that chemokine receptor profiling might be a novel tool for discriminating ANKL cells from benign NK cells.

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Aggressive Granular Lymphocyte Leukemia of Natural Killer Cell Type in an Elderly Patient.

Cited by 2 publications

References 15 publications

Aggressive NK-Cell Leukemia

Aggressive NK-Cell Leukemia

Significance of chemokine receptor expression in aggressive NK cell leukemia

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