title: Transient spontaneous remission in congenital MLL-AF10 rearranged acute myeloid leukemia presenting with cardiorespiratory failure and meconium ileus.
abstract: BACKGROUND
  Neonatal leukemia is a rare disease with an estimated prevalence of about one to five in a million neonates. The majority being acute myeloid leukemia (AML), neonatal leukemia can present with a variety of symptoms including hyperleucocytosis, cytopenia, hepatosplenomegaly, and skin infiltrates. Chromosomal rearrangements including mixed lineage leukemia (MLL) translocations are common in neonatal AML.
  
  
  METHODS
  A female neonate born at 34 weeks gestation presented with cardiorespiratory failure, hepatosplenomegaly, pancytopenia, and coagulopathy. She required intensive care treatment including mechanical ventilation, high-dose catecholamine therapy, and multiple transfusions. Small intestinal biopsy obtained during laparotomy for meconium ileus revealed an infiltrate by an undifferentiated monoblastic, MLL-rearranged leukemia. No other manifestations of leukemia could be detected. After spontaneous clinical remission, lasting 5 months without any specific treatment, the patient presented with leukemia cutis and full-blown monoblastic leukemia. MLL-AF10-rearranged AML could be re-diagnosed and successfully treated with chemotherapy and hematopoietic stem cell transplantation.
  
  
  CONCLUSIONS
  Our patient exhibited a unique manifestation of neonatal MLL-AF10 rearranged AML with cardiorespiratory failure and intestinal infiltration. It highlights the importance of leukemia in the differential diagnosis of neonatal distress, congenital hematological abnormalities, and skin lesions.
authors: Medical Faculty, Department of General Pediatrics, Neonatology and Pediatric Cardiology, Centre for Child and Adolescent Health, University of Duesseldorf, Duesseldorf, Germany.
authors: Tobias
authors: T
authors: Gyárfás
authors: Department of Neonatology, Staedtische Kliniken Moenchengladbach, Elisabeth Krankenhaus Rheydt, Rheydt, Germany.
authors: Juergen
authors: J
authors: Wintgens
authors: Department of Neonatology, Staedtische Kliniken Moenchengladbach, Elisabeth Krankenhaus Rheydt, Rheydt, Germany.
authors: Wolfgang
authors: W
authors: Biskup
authors: Department of Pathology, Christian-Albrechts-University Kiel, Kiel, Germany.
authors: Ilske
authors: I
authors: Oschlies
authors: Department of Pathology, Christian-Albrechts-University Kiel, Kiel, Germany.
authors: Wolfram
authors: W
authors: Klapper
authors: Institute of Human Genetics, Christian-Albrechts-University Kiel, Kiel, Germany.
authors: Reiner
authors: R
authors: Siebert
authors: Institute of Human Genetics, Christian-Albrechts-University Kiel, Kiel, Germany.
authors: Susanne
authors: S
authors: Bens
authors: MLL Munich Leukemia Laboratory, Munich, Germany.
authors: Claudia
authors: C
authors: Haferlach
authors: Department of Pediatric Oncology, Hematology and Clinical Immunology, University Children's Hospital, Medical Faculty, Heinrich Heine University, Moorenstr. 5, 40225, Duesseldorf, Germany.
authors: Roland
authors: R
authors: Meisel
authors: Department of Pediatric Oncology, Hematology and Clinical Immunology, University Children's Hospital, Medical Faculty, Heinrich Heine University, Moorenstr. 5, 40225, Duesseldorf, Germany. michaela.kuhlen@med.uni-duesseldorf.de.
authors: Michaela
authors: M
authors: Kuhlen
authors: Department of Pediatric Oncology, Hematology and Clinical Immunology, University Children's Hospital, Medical Faculty, Heinrich Heine University, Moorenstr. 5, 40225, Duesseldorf, Germany.
authors: Arndt
authors: A
authors: Borkhardt
keywords: Acute myeloid leukemia; Cardiorespiratory failure; Case report; Congenital; Meconium ileus; Spontaneous remission