We report a patient with infant leukemia showing the rare phenotypes of CD2+, CD4+, CD7+, CD56+, CD3–, CD5– who followed an unusual clinical course. The patient was a 3-month-old girl who was admitted because of unusual purpura looking like a black ring. On admission WBC count was 85.8 × 109/l and bone marrow aspiration revealed a nucleated cell count of 112.0 × 109/l with 70.8% atypical lymphocytes. On the 3rd hospital day, the WBC count decreased by about 1/5 without chemotherapy and partial remission was obtained. But about 3 weeks later, the WBC count increased again and she died. Based on surface marker analysis, genotypic analysis and autopsy, we diagnosed infant leukemia suggestive of natural killer (NK) cell precursor origin.

Fernandez LA, Pope B, Lee C, Zayed E: Aggressive natural killer cell leukemia in an adult with establishment of an NK cell line. Blood 1986;67:925–930.
Nakamura S, Suchi T, Koshikawa T, Koshikawa T, Kitoh K, Koike K, Komatsu H, Iida S, Kagami Y, Ogura M, Katoh E, Kurita S, Suzuki H, Kobashi Y, Yamabe H, Hirabayashi N, Ueda R, Takahashi T: Clinicopathologic study of CD56 (NCAM)-positive angiocentric lymphoma occurring in sites other than the upper and lower respiratory tract. Am J Surg Pathol 1995;19:284–296.
Kobayashi Y, Nakamura S, Sasajima Y, Koshikawa T, Yatabe Y, Kitoh K, Mori S, Ueda R, Yamabe H, Suchi T: Inconsistent association of Epstein-Barr virus with CD56 (NCAM)-positive angiocentric lymphoma occurring in sites other than the upper and lower respiratory tract. Histopathology 1996;28:111–120.
Suzuki R, Yamamoto K, Seto M, Kagami Y, Ogura M, Yatabe Y, Suchi T, Kodera Y, Morishima Y, Takahashi T, Saito H, Ueda R, Nakamura S: CD7+ and CD56+ myeloid/natural killer cell precursor acute leukemia: A distinct hematolymphoid disease entity. Blood 1997;90:2417–2428.
Rosenbloom D, Gilbert R: Reversible flu-like syndrome, leukopenia, and thrombocytopenia induced by allupurinol. Drug Intell Clin Pharm 1981;15:286–287.
Anderson J, Ernberg I: Management of Epstein-Barr virus infections. Am J Med 1988;85:107–115.
Imamura N, Kusunoki Y, Kawa-Ha K, Yumura K, Oda K, Abe K, Dohy H, Inada T, Kajihara H, Kuramoto A: Aggressive natural killer cell leukemia/lymphoma: Report of four cases and review of the literature. Br J Haematol 1990;75:49–59.
Thomas P, Loughran JR: Clonal disease of large granular lymphocytes. Blood 1993;82:1–4.
Chan JKC, Sin VC, Wong KF, Ng CS, Tsang WYW, Chan CH, Cheung MMC, Lau WH: Nonnasal lymphoma expressing the natural killer cell marker CD56: A clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood 1997;89:4501–4513.
Suzuki R, Nakamura S: Malignancies of natural killer (NK) cell precursor: myeloid/NK cell precursor acute leukemia and blastic NK cell lymphoma/leukemia. Leuk Res 1999;23:615–624.
Koizumi S, Seki H, Tachinami T, Taniguchi M, Matsuda A, Taga K, Nakarai T, Kato E, Taniguchi N, Nakamura H: Malignant clonal expansion of large granular lymphocytes with a Leu11+, Leu7– surface phenotype: In vitro responsiveness of malignant cells to recombinant human interleukin 2. Blood 1986;68:1065–1073.
Kawa-Ha K, Ishihara S, Ninomiya T, Yumura-Yagi K, Hara J, Murayama F, Tawa A, Hirai K: CD3-negative lymphoproliferative disease of granular lymphocytes containing Epstein-Barr virus DNA. J Clin Invest 1989;84:51–55.
Ichikawa M, Kawai H, Komiyama A, Tsudo M, Miyasaka M, Kinoshita A, Nakazawa S: Functional p75 interleukin-2 receptor expression on the fresh blast cells in childhood acute lymphoblastic leukemia with natural killer cell properties. Am J Hematol 1991;36:259–264.
Gardiner CM, Reen DJ, O’Meara A: Recognition of unusual presentation of natural killer cell leukemia. Am J Hematol 1995;50:133–139.
Teshima T, Miyaji R, Fukuda M, Ohshima K: Bone-marrow transplantation for Epstein-Barr-virus-associated natural killer cell-large granular lymphocyte leukemia. Lancet 1996;347:1124.
Ohnuma K, Toyoda Y, Nishihira H, Iguchi A, Honda K, Nagao T, Kigasawa H, Tanaka Y, Kikuta H: Aggressive natural killer (NK) cell lymphoma: Report of a pediatric case and review of the literature. Leuk Lymphoma 1997;25:387–392.
Cho KH, Kim CW, Kwon OS, Yang SG, Park KC, Park MH, Cho HI, Kim JG: Epstein-Barr virus-associated lymphoproliferative eruption with progression to large granular lymphocytic leukemia. Br J Dermatol 1997;137:426–430.
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