Advances in the management and monitoring of extranodal NK/T‐cell lymphoma, nasal type

Abstract

Summary Extranodal natural killer (NK)/T‐cell lymphoma, nasal type, has a unique geographic distribution. Its pathology is characterized by marked angio‐invasion and tissue necrosis. A typical NK‐cell phenotype is usually present: CD2+, CD3 epsilon+, CD56+, cytotoxic molecules+ and Epstein–Barr virus (EBV)+. Magnetic Resonance Imaging helps to clearly define the local involvement. Positron Emission Tomography helps to demonstrate system spread. Various prognostic variables (International Prognostic Index or the Korean Prognostic Index) should be documented. This may include quantification of plasma EBV DNA. For localized nasal disease, radiotherapy is important, although chemotherapy is often added. Sustainable remission is observed in over half of these patients. For extra‐nasal or disseminated disease, systemic chemotherapy becomes the mainstay and the prognosis is usually poor. Doxorubicin‐containing regimens are not entirely satisfactory and l‐asparaginase containing regimens are being investigated. Patients with poor prognostic features may be considered for an early autologous haematopoietic stem cell transplant. Allogenetic transplantation is efficacious but is associated with high transplant‐related mortality.

Racial patterns of extranodal natural killer/T‐cell lymphoma, nasal type, in California: a population‐based study

“…Radiotherapy as the sole modality for localized disease results in overall response rates of 60-80%, complete response rates of 40-80%, and a 5-year OS of approximately 50% (Kwong, 2005;Liang, 2009). Unfortunately, up to 50% of patients relapse either in-field or systemically (Au, 2010).…”

Current and future management of NK/T-cell lymphoma based on clinical trials

“…Several comprehensive reviews on the pathophysiology, diagnosis, clinical features, risk stratification, and prognosis of ENKL have already been published [5][6][7][8][9]. This review will focus on the evidence from prospective clinical trials for ENKL and discuss the most recommended management at present and future directions for the better management of ENKL.…”

Classical Hodgkin Lymphoma

“…As mentioned early, L-asparaginase is not affected by P-glycoprotein. Furthermore, the tumor cells lack Lasparagine synthetase and are susceptible to L-asparaginase which depletes L-asparagines in NK lymphoma cells [14]. A retrospective analysis of Chinese patients with relapsed or refractory NK/T-cell lymphoma, nasal type yielded an overall response rate of 82% and a CR rate of 56%.…”

“…For the stage III/IV nasal NK/T-cell lymphomas, extran-asal NK/T-cell lymphomas and aggressive NK-cell lymphoma/ leukemia, chemotherapy is the primary treatment [13,14]. Results from chemotherapy adopted from treating aggressive B-cell lymphomas such as CHOP is far from satisfactory [14].…”

“…Patients with IPI of 1 or less were shown to have a better overall survival [13,14]. In a recent retrospective analysis of 172 patients with extranodal NK/Tcell lymphoma, nasal type, and aggressive NK-cell leukemia, 4 prognostic factors were identified.…”