Spontaneous remission of infantile acute nonlymphocytic leukemia for 11 years in a child with normal karyotype

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Abstract
Background. Leukemia or a leukemia-like picture in phenotypically normal infants with a normal marrow karyotype who undergo spontaneous remission is extremely unusual. Results. A 6–week-old, phenotypically normal boy first was seen in 1979 with hepatosplenomegaly, a 36 × 109/1 leukocyte count, a leukoerythroblastic picture, and increased blasts in the bone marrow aspirate. Because no other known causes of leukemoid reaction could be demonstrated, the picture was consistent at that time with acute nonlymphocytic leukemia (ANLL), French-American-British (FAB) Ml; however, because of the age of the child, the low blast count, normal cytogenetics, and questions about this diagnosis, treatment was withheld. Hematologic laboratory values normalized by the time he was 1 year of age. Eleven years later, he presented a similar picture that rapidly progressed to overt leukemia (FAB Ml). A normal marrow karyotype was consistent throughout the 11 years of follow-up. Treatment of ANLL was initiated, resulting in marrow remission. This was followed by allogeneic bone marrow transplantation. He had a relapse again and was retreated, achieving a second marrow remission, and had a second allogeneic bone marrow transplantation. However, the patient died of venoocclusive disease of the liver after the second transplant. Conclusion. The diagnosis and prognosis of infants with an abnormal hematologic picture similar to ANLL but who are phenotypically and karyotypically normal are not as straightforward as in the older child or adult. Treatment should be withheld in this setting until the leukemic process is overt.