Juvenile xanthogranuloma (JXG), one of the most common forms of non-Langerhans cell histiocytosis (LCH), usually presents in young children as spontaneously regressing cutaneous lesions. However, the systemic type of JXG is difficult to treat in newborn infants, and fatal cases have been reported. In the patient described here, solid masses were discovered by fetal sonography during the 38th gestational week. At birth she had multiple tumors on the back, cheek, and hip as well as marked hepatosplenomegaly accompanied by respiratory failure. Laboratory results indicated pancytopenia, obstructive liver dysfunction, and coagulopathy. Brain magnetic resonance imaging revealed a tumor at the left pontine angle, and dysmorphic histiocytes were present in her spinal fluid. She was diagnosed with systemic JXG by histopathologic findings of the hip mass. The LCH-based multiagent chemotherapy including cytarabine, vincristine, methotrexate, and prednisolone ameliorated the symptoms rapidly. She was treated for 12 months and is currently doing well as a normally developing 2-year-old.

BACKGROUND.L‐asparaginase is a key drug in the treatment of childhood acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL). However, L‐asparaginase can cause a fatal complication of pancreatitis, and an effective treatment for L‐asparaginase–associated pancreatitis (AAP) has not been developed to date. The authors investigated whether rapidly treating children with AAP by continuous regional arterial infusion (CRAI) of protease inhibitor and antibiotic would quickly resolve AAP.METHODS.Between 2000 and 2007, 104 pediatric patients with ALL or LBL were treated at the authors' affiliated hospitals with intensive regimens that included Escherichia coli‐derived L‐asparaginase. Six of 104 patients developed severe AAP. One patient was treated with intravenous infusion of protease inhibitor, and the remaining 5 patients received CRAI of protease inhibitor and antibiotic within 48 hours of the onset of AAP.RESULTS.The patient who received intravenous protease inhibitor had pseudocyst formation and developed a subsequent leukemic recurrence after the interruption of chemotherapy for 4.5 months. In the other patients, AAP subsided within 2 to 6 days after the start of CRAI, and serious complications did not emerge. Significantly, chemotherapy could be resumed within 4 weeks (range, 12‐23 days) afterthe onset of AAP, and the patients were in complete remission from 4 months to 44 months with further chemotherapy that excluded L‐asparaginase.CONCLUSIONS.The current results indicated that early introduction of CRAI of protease inhibitor and antibiotic is suitable for treating severe AAP. Cancer 2008. © 2008 American Cancer Society.

No abstract

No abstract

No abstract