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Hematology Reports
Volume 4
Issue 3
10.4081/hr.2012.e16
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Hematology Reports is published by MDPI from Volume 14 Issue 1 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Case Report

Epstein-Barr Virus-Negative Aggressive Natural Killer-Cell Leukaemia with High P-Glycoprotein Activity and Phosphorylated Extracellular Signal-Regulated Protein Kinases 1 and 2

by
Sanja Perkovic
1,*,
Sandra Basic-Kinda
2,
Vladimir Gasparovic
3,
Zeljko Krznaric
4,
Jaksa Babel
3,
Ivana Ilic
5,
Igor Aurer
2 and
Drago Batinic
1
1
Department of Laboratory Diagnostics, Division of Immunology and Department of Internal Medicine, University Hospital Centre Zagreb and School of Medicine, University of Zagreb, Kispaticeva 12, HR-10000 Zagreb, Croatia
2
Division of Haematology, University Hospital Centre Zagreb and School of Medicine, University of Zagreb, HR-10000 Zagreb, Croatia
3
Division of Intensive Care Medicine, University Hospital Centre Zagreb and School of Medicine, University of Zagreb, HR-10000 Zagreb, Croatia
4
Division of Gastroenterology, University Hospital Centre Zagreb and School of Medicine, University of Zagreb, HR-10000 Zagreb, Croatia
5
Department of Pathology and Cytology, Division of Gastroenterology, University Hospital Centre Zagreb and School of Medicine, University of Zagreb, HR-10000 Zagreb, Croatia
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2012, 4(3), e16; https://doi.org/10.4081/hr.2012.e16
Submission received: 6 March 2012 / Revised: 22 May 2012 / Accepted: 12 June 2012 / Published: 4 September 2012

Abstract

Aggressive natural killer-cell leukaemia (ANKL) is a rare type of disease with fulminant course and poor outcome. The disease is more prevalent among Asians than in other ethnic groups and shows strong association with Epstein-Barr virus (EBV) and P-glycoprotein (P-gp) expression associated with multidrug resistance. Here we present a case of a 47 year old Caucasian female with a prior medical history of azathioprine treated ulcerative colitis who developed EBV-negative form of ANKL. The patient presented with hepatosplenomegaly, fever and nausea with peripheral blood and bone marrow infiltration with up to 70% of atypical lymphoid cells positive for cCD3, CD2, CD7, CD56, CD38, CD45, TIA1 and granzyme B, and negative for sCD3, CD4, CD5, CD8, CD34 and CD123 indicative of ANKL. Neoplastic CD56+ NK-cells showed high level of P-glycoprotein expression and activity, but also strong expression of phosphorylated extracellular signal-regulated protein kinases 1 and 2 (ERK1/2) MAP kinase. The patient was treated with an intensive polychemotherapy regimen designed for treatment of acute lymphoblastic leukaemia, but one month after admission developed sepsis, coma and died of cardiorespiratory arrest. We present additional evidence that, except for the immunophenotype, leukaemic NK-cells resemble normal NK-cells in terms of P-gp functional capacity and expression of phosphorylated ERK1/2 signalling molecule. In that sense drugs that block P-glycoprotein activity and activated signalling pathways might represent new means for targeted therapy.
Keywords: NK-cell leukaemia; P-glycoprotein; ERK MAP kinase; ulcerative colitis NK-cell leukaemia; P-glycoprotein; ERK MAP kinase; ulcerative colitis

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MDPI and ACS Style

Perkovic, S.; Basic-Kinda, S.; Gasparovic, V.; Krznaric, Z.; Babel, J.; Ilic, I.; Aurer, I.; Batinic, D. Epstein-Barr Virus-Negative Aggressive Natural Killer-Cell Leukaemia with High P-Glycoprotein Activity and Phosphorylated Extracellular Signal-Regulated Protein Kinases 1 and 2. Hematol. Rep. 2012, 4, e16. https://doi.org/10.4081/hr.2012.e16

AMA Style

Perkovic S, Basic-Kinda S, Gasparovic V, Krznaric Z, Babel J, Ilic I, Aurer I, Batinic D. Epstein-Barr Virus-Negative Aggressive Natural Killer-Cell Leukaemia with High P-Glycoprotein Activity and Phosphorylated Extracellular Signal-Regulated Protein Kinases 1 and 2. Hematology Reports. 2012; 4(3):e16. https://doi.org/10.4081/hr.2012.e16

Chicago/Turabian Style

Perkovic, Sanja, Sandra Basic-Kinda, Vladimir Gasparovic, Zeljko Krznaric, Jaksa Babel, Ivana Ilic, Igor Aurer, and Drago Batinic. 2012. "Epstein-Barr Virus-Negative Aggressive Natural Killer-Cell Leukaemia with High P-Glycoprotein Activity and Phosphorylated Extracellular Signal-Regulated Protein Kinases 1 and 2" Hematology Reports 4, no. 3: e16. https://doi.org/10.4081/hr.2012.e16

APA Style

Perkovic, S., Basic-Kinda, S., Gasparovic, V., Krznaric, Z., Babel, J., Ilic, I., Aurer, I., & Batinic, D. (2012). Epstein-Barr Virus-Negative Aggressive Natural Killer-Cell Leukaemia with High P-Glycoprotein Activity and Phosphorylated Extracellular Signal-Regulated Protein Kinases 1 and 2. Hematology Reports, 4(3), e16. https://doi.org/10.4081/hr.2012.e16

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