Favorable outcome in a child with EBV-negative aggressive NK cell leukemia
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CASE REPORT
Favorable outcome in a child with EBV-negative aggressive NK cell leukemia Jeong A. Park • Kyung Ran Jun • So Hyun Nam Thad T. Ghim
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Received: 6 December 2012 / Revised: 15 March 2013 / Accepted: 21 March 2013 / Published online: 4 April 2013 Ó The Japanese Society of Hematology 2013
Abstract Aggressive natural killer cell leukemia (ANKL) is a rare malignant disorder of mature NK cells frequently associated with Epstein–Barr virus (EBV). This malignancy is typically treated with intensive remission induction chemotherapy followed by allogeneic hematopoietic stem cell transplantation (HSCT). EBV-negative ANKL and childhood ANKL, however, are not well defined and the optimal therapeutic strategy in these cases is poorly understood. Here, we present a unique pediatric EBV-negative ANKL patient who achieved a successful treatment outcome after intensified ALL type chemotherapy without allogeneic HSCT. Keywords Pediatric Epstein–Barr virus Aggressive NK cell leukemia Chemotherapy
Introduction Aggressive natural killer cell leukemia (ANKL) is a very rare malignant lymphoproliferative disorder that is usually refractory to chemotherapy resulting in poor outcome [1, 2]. Since most ANKL cases that occur in adults are infected with EBV, the EBV infection is deemed to play an important role in the development of ANKL and might be a significant determinant for its aggressive clinical course as well. Although rare, the existence of EBV-negative ANKL is also well recognized, and reports suggest that the clinical features of this EBV-negative disease differ from EBVpositive ANKL [3]. In this study, we review the literature of ANKL in general, and describe an EBV-negative pediatric ANKL patient whom we had treated with chemotherapy and showed an excellent treatment outcome.
Case report J. A. Park (&) Department of Pediatrics, Haeundae Paik Hospital, Inje University College of Medicine, 1435 Jwa-dong, Haeundae-gu, Busan 612-862, Korea e-mail: [email protected] K. R. Jun Department of Diagnostic Laboratory Medicine, Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea S. H. Nam Department of Surgery, Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea T. T. Ghim Formerly Pediatric Oncology Center, Korean National Cancer Center, Ilsan, Gyunggido, Korea
In June 2010, a 14-year-old girl presented with fever, fatigue, and right thigh pain. She had marked hepatosplenomegaly and multiple lymphadenopathies. There were no skin lesions. Blood counts showed anemia (hemoglobin 8.7 g/dL), leukopenia (white blood cell count 3.56 9 109/L), and thrombocytopenia (platelets 83 9 109/ L). A peripheral blood smear revealed 15 % undifferentiated blasts. Blood chemistry showed normal range of total bilirubin (0.6 mg/dL) and mild elevation of aspartate aminotransferase (51 IU/L) and alanine aminotransferase (83 IU/L). Blood urea nitrogen was 17 mg/dL and creatinine was 0.87 mg/dL. Lactate dehydrogenase was 589 IU/ L, uric acid was 6.2 mg/dL, and level of ferritin was 969 ng/mL. Routine b
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