Extranodal natural killer/T-cell lymphoma in Malawi: a report of three cases

• Published in: BMC cancer Vol. 17; no. 1; pp. 633 - 4
• Main Authors: Tomoka, Tamiwe, Powers, Eric, van der Gronde, Toon, Amuquandoh, Amy, Dhungel, Bal Mukunda, Kampani, Coxcilly, Kamiza, Steve, Montgomery, Nathan D., Fedoriw, Yuri, Gopal, Satish
• Format: Journal Article
• Language: English
• Published: London BioMed Central 06.09.2017; BioMed Central Ltd; BMC
• Subjects: Adult; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biomarkers; Biomedical and Life Sciences; Biomedicine; Biopsy; Cancer; Cancer Research; Case Report; CD20 antigen; CD3 antigen; CD56 antigen; Chemotherapy; Children; Classification; Cyclophosphamide; Cyclophosphamide - therapeutic use; Dehydrogenases; Deoxyribonucleic acid; Development and progression; DNA; DNA, Viral; Dosage and administration; Doxorubicin; Doxorubicin - therapeutic use; Epstein-Barr virus; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - virology; Familiarity; Female; Gangrene; Health Promotion and Disease Prevention; Herpesvirus 4, Human - genetics; Humans; immunity and cancer vaccines; Immunophenotyping - methods; Immunoproliferative diseases; Infection; Laboratories; Lymphocytes T; Lymphoma; Lymphoma, Extranodal NK-T-Cell - diagnosis; Lymphoma, Extranodal NK-T-Cell - drug therapy; Lymphoma, Extranodal NK-T-Cell - etiology; Lymphoma, Extranodal NK-T-Cell - metabolism; Malawi; Male; Medicine/Public Health; Middle Aged; Natural killer cells; Non-Hodgkin lymphoma; Non-Hodgkin's lymphomas; Oncology; Plasma; Prednisone; Prednisone - therapeutic use; Proteins; Radiation therapy; Ribonucleic acid; RNA; Sub-Saharan Africa; Surgical Oncology; T-cell lymphoma; Treatment Outcome; Tumors; Vincristine; Vincristine - therapeutic use; Malawi; Southeast Asia; Non-Hodgkin lymphoma; Epstein-Barr virus; Sub-Saharan Africa
• ISSN: 1471-2407; 1471-2407
• DOI: 10.1186/s12885-017-3612-y

Background Extranodal NK/T-cell lymphoma (ENKTCL) reports from sub-Saharan Africa (SSA) are remarkably rare, despite early childhood acquisition and high prevalence of the causative infectious agent, Epstein-Barr virus (EBV), and frequent occurrence of other lymphoproliferative disorders causally associated with EBV. Case presentations At a national teaching hospital in Malawi, three patients of African descent were seen with ENKTCL between 2013 and 2014. Patients were aged between 29 and 60 years, two with craniofacial involvement and one with a primary abdominal tumor, and all were HIV-negative. All had systemic B symptoms, and two severely impaired performance status. On histologic review, morphology and immunophenotyping demonstrated classical ENKTCL features in all cases, including diffuse proliferations of intermediate-to-large atypical lymphocytes with high mitotic activity and extensive background necrosis, positivity for CD3 and CD56, and negativity for CD20. By in situ hybridization, all three tumors were positive for EBV-encoded RNA (EBER). Baseline plasma EBV DNA was also markedly elevated for all three patients. Due to radiotherapy and chemotherapy limitations, patients were treated with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) with rapid disease progression. All three patients died from progressive lymphoma within 3 months of initial diagnosis. Conclusions Our experience with these three patients in Malawi can highlight that ENKTCL does indeed occur in SSA, increase familiarity with ENKTCL among clinicians and pathologists throughout the region, and emphasize the need for better diagnosis and treatment for this neglected population.