Treatment of extranodal NK/T cell lymphoma, nasal type
- Motoko Yamaguchi, MD, PhD
Motoko Yamaguchi, MD, PhD
- Assistant Professor, Department of Hematology and Oncology
- Mie University Graduate School of Medicine
Extranodal natural killer/T cell lymphoma, nasal type (ENKL; formerly called angiocentric lymphoma), is the most common cause of the syndrome known as "lethal midline granuloma." It is an extranodal lymphoma, usually with a natural killer (NK) cell phenotype and positivity for Epstein-Barr virus (EBV), with a broad morphologic spectrum, frequent necrosis, and angioinvasion [1,2]. It is designated NK/T because most cases have a natural killer cell origin, but a small minority is derived from cytotoxic T cells. (See "Classification of the hematopoietic neoplasms".)
The treatment of ENKL will be discussed here. The clinical presentation, pathologic features, and diagnosis are presented separately. (See "Clinical manifestations, pathologic features, and diagnosis of extranodal NK/T cell lymphoma, nasal type".)
The initial evaluation of patients with non-Hodgkin lymphoma (NHL) must establish the precise histologic subtype, the extent and sites of disease (table 1), and the performance status (table 2A-B) of the patient. General approaches to the diagnostic work-up and staging of NHL are presented separately. (See "Clinical presentation and diagnosis of non-Hodgkin lymphoma" and "Evaluation, staging, and response assessment of non-Hodgkin lymphoma".)
Once the diagnosis has been definitively established, the pretreatment evaluation determines both the bulk of disease and the individual's comorbidities that are likely to have an impact on treatment options. In addition to a history and physical examination, it is our practice to perform the following pretreatment studies in patients with ENKL:
●Laboratory studies include a complete blood count with differential, chemistries with liver and renal function and electrolytes, lactate dehydrogenase (LDH), hepatitis B virus, HIV, and uric acid. Measurement of a pretreatment plasma EBV DNA by quantitative polymerase chain reaction, if available, helps to predict prognosis and serves as a baseline value with which to compare during response assessment [3,4]. (See "Hepatitis B virus reactivation associated with immunosuppressive therapy" and 'Response assessment' below and 'Prognosis' below.)
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- PRETREATMENT EVALUATION
- INDUCTION THERAPY
- Localized disease
- Disseminated disease
- Central nervous system prophylaxis
- CONSOLIDATION THERAPY
- Hematopoietic cell transplantation
- PATIENT FOLLOW-UP
- Response assessment
- Reconstructive surgery
- Surveillance for relapse
- TREATMENT OF RELAPSED OR REFRACTORY DISEASE
- Allogeneic HCT
- PD-1 blockade
- CLINICAL TRIALS
- SUMMARY AND RECOMMENDATIONS