Aggressive Natural Killer Cell Leukemia/Lymphoma: Case Report, use of Telesynergy™ and Review of the Literature

Murdock, Joanne; Jaffe, Elaine S.; Wilson, Wyndham H.; McManus, Damian; Alexander, H. Denis; Morris, T. C. M.

doi:10.1080/10428190310001646879

Cited by 13 publications

(5 citation statements)

References 20 publications

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“…21 Rare sporadic cases occur in Western populations. 36 There had been uncertainty as to whether ANKL, similar to extranodal NK/T lymphoma, is an EBV-associated disorder. Recent studies have confirmed positivity for EBV in both endemic and nonendemic populations.…”

Section: Aggressive Nk-cell Leukemiamentioning

confidence: 99%

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The Pathology of NK-Cell Lymphomas and Leukemias

Nava

Jaffe

2005

Advances in Anatomic Pathology

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Natural killer (NK) cell lymphomas and leukemias are a rare but clinically important group of neoplasms. Most of these tumors are aggressive, with a high rate of mortality. They include extranodal NK/T-cell lymphomas of nasal type and aggressive NK-cell leukemias. Both are Epstein-Barr virus (EBV) associated and show similar epidemiologic features. A closely related entity seen mainly in children is hydroa vacciniforme-like lymphoma, which also is EBV positive. EBV influences the pathophysiology of these tumors, through the induction of cytokines and chemokines. The differential diagnosis of NK-cell malignancies includes fulminant EBV-associated T-cell lymphoproliferative disorder, a condition referred to in the past as fatal infectious mononucleosis. Benign proliferations of NK cells can be seen in association with viral infection. The disease formerly referred to as blastic NK-cell lymphoma is now considered to be a malignancy derived from a dendritic cell precursor.

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Section: Aggressive Nk-cell Leukemiamentioning

confidence: 99%

“…Recent studies have confirmed positivity for EBV in both endemic and nonendemic populations. 34,[36][37][38] Despite similarities in phenotype and genotype, clinical and morphologic features distinguish these two disorders. 39 First, ANKL is an aggressive disorder with a poor prognosis, irrespective of treatment.…”

Section: Aggressive Nk-cell Leukemiamentioning

confidence: 99%

The Pathology of NK-Cell Lymphomas and Leukemias

Nava

Jaffe

2005

Advances in Anatomic Pathology

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“…Comparatively, based on the Japanese survey of NK-cell neoplasms diagnosed from 1994 to 1998 [36], the NK-cell Tumor Study Group reported on a Japanese series of 172 NK-cell tumors, which included 22 ANKCL (12.8%) [37]. Few European series of NKTCL were published to date [38,39] and, in Europe, reports on ANKCL are limited to sporadic cases [40-43]. …”

Section: Reviewmentioning

confidence: 99%

Aggressive mature natural killer cell neoplasms: from epidemiology to diagnosis

Lima

2013

Orphanet J Rare Dis

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Mature natural killer (NK) cell neoplasms are classified by the World Health Organization into NK/T cell lymphoma, nasal type (NKTCL), aggressive NK-cell leukemia (ANKCL) and chronic lymphoproliferative disorders of NK-cells, the latter being considered provisionally. NKTCL and ANKCL are rare diseases, with higher prevalence in Asia, Central and South America. Most NKTCL present extranodal, as a destructive tumor affecting the nose and upper aerodigestive tract (nasal NKTCL) or any organ or tissue (extranasal NKTCL) whereas ANKCL manifests as a systemic disease with multiorgan involvement and naturally evolutes to death in a few weeks. The histopathological hallmark of these aggressive NK-cell tumors is a polymorphic neoplastic infiltrate with angiocentricity, angiodestruction and tissue necrosis. The tumor cells have cytoplasmatic azurophilic granules and usually show a CD45+bright, CD2+, sCD3-, cytCD3epsilon+, CD56+bright, CD16−/+, cytotoxic granules molecules+ phenotype. T-cell receptor genes are in germ-line configuration. Epstein-Barr virus (EBV) -encoded membrane proteins and early region EBV RNA are usually detected on lymphoma cells, with a pattern suggestive of a latent viral infection type II. Complex chromosomal abnormalities are frequent and loss of chromosomes 6q, 11q, 13q, and 17p are recurrent aberrations. The rarity of the NK-cell tumors limits our ability to standardize the procedures for the diagnosis and clinical management and efforts should be made to encourage multi-institutional registries.

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“…Successful treatment with alemtuzumab has been reported in the subtypes of mature T-cell neoplasms, such as Lennert's lymphoma [31], hepatosplenic gammadelta T-cell lymphoma [32], angioimmunoblastic T-cell lymphoma [33] and primary cutaneous CD30-negative CD8-positive cytotoxic large T-cell lymphoma [34]. Data of its application in aggressive NK-cell leukemia/lymphoma is scanty with possible potential efficacy [35,36]. However, its combination with chemotherapy is more likely leading to more sustain and higher response rate in view of the aggressiveness of the disease.…”

Section: Introductionmentioning

confidence: 99%

The Outcome of HyperCVAD Combined with Alemtuzumab for the Treatment of Aggressive T-Cell and NK-Cell Neoplasms

Kuan

Chang

Lau

et al. 2011

Indian J Hematol Blood Transfus

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We report our experience in using six cycles of hyperCVAD in combination with alemtuzumab for the treatment of aggressive T-cell and NK/T-cell neoplasms. Seven females and six males with the median age of 41 (range 18-60) diagnosed with T-cell acute lymphoblastic lymphoma and peripheral T-cell and NK/T-cell neoplasms (n PTCL = 6, n T-cell ALL = 3, n NK/T-cell neoplasms = 4) from 2006 to 2008 were treated with alemtuzumab-hyperCVAD regimen. A total of nine patients (69%) responded to the regimen, with seven achieved complete remission and two achieved partial remission. The median progression free survival and overall survival duration among the responders with complete remission were 12.9 and 24.9 months respectively. The incidence of relapse among the responders was 44% and the overall survival rate was 23%. Only four (31%) patients completed the six cycles of alemtuzumab-hyperCVAD. Others were stopped earlier due to progressive disease (n = 2), cytomegalovirus (CMV) reactivation and/or disease (n = 3), death not due to disease (n = 2), and patient's refusal to continue alemtuzumab (n = 2). The incidence of death not due to disease, CMV reactivation and recurrent CMV reactivation were 50, 50 and 17%, respectively. This study shows that alemtuzumab in combination with hyperCVAD regimen is a feasible regimen but with high toxicity. The toxicity might be reduced with the incorporation of filgrastim and use of valganciclovir as CMV prophylaxis.

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Aggressive Natural Killer Cell Leukemia/Lymphoma: Case Report, use of Telesynergy™ and Review of the Literature

Cited by 13 publications

References 20 publications

The Pathology of NK-Cell Lymphomas and Leukemias

The Pathology of NK-Cell Lymphomas and Leukemias

Aggressive mature natural killer cell neoplasms: from epidemiology to diagnosis

The Outcome of HyperCVAD Combined with Alemtuzumab for the Treatment of Aggressive T-Cell and NK-Cell Neoplasms

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