Cytosine Arabinoside and Mitoxantrone Followed by Second Allogeneic Transplant for the Treatment of Children With Refractory Juvenile Myelomonocytic Leukemia
| Autor: | Peter F. Coccia, Phyllis I. Warkentin, Warren G. Sanger, James L. Wisecarver, Sachit Patel, James Harper, Bruce Geoffrey Gordon, Don W. Coulter, Alfred C. Grovas |
|---|---|
| Rok vydání: | 2014 |
| Předmět: |
Male
Antimetabolites Antineoplastic medicine.medical_specialty Transplantation Conditioning medicine.medical_treatment Hematopoietic stem cell transplantation Philadelphia chromosome Gastroenterology Disease-Free Survival Monocytosis Recurrence Internal medicine medicine Humans Transplantation Homologous Leukocytosis Child JMML Transplantation Chimera Juvenile myelomonocytic leukemia business.industry Cytarabine Hematopoietic Stem Cell Transplantation Infant Original Articles Hematology allogeneic transplant medicine.disease Combined Modality Therapy Tissue Donors PTPN11 surgical procedures operative chimerism Leukemia Myelomonocytic Juvenile Oncology Child Preschool Retreatment Pediatrics Perinatology and Child Health Immunology Noonan syndrome Mitoxantrone medicine.symptom business medicine.drug |
| Zdroj: | Journal of Pediatric Hematology/Oncology |
| ISSN: | 1077-4114 |
| DOI: | 10.1097/mph.0000000000000077 |
| Popis: | Juvenile myelomonocytic leukemia (JMML) is a rare mixed myelodysplastic and myeloproliferative clonal disorder of early childhood representing about 1% to 2% of all pediatric leukemias.1–3 Most cases present before 6 years of age, with a median age of 2 years. Clinical presentation includes pallor, rash, hepatomegaly, splenomegaly, lymphadenopathy, and recurrent infections.2–5 JMML has an increased incidence in patients with neurofibromatosis and Noonan syndrome as well as genetic mutations in the RAS signaling pathway, PTPN11, CBL, and NF1 gene.6–10 Although most children demonstrate a normal karyotype, monosomy 7 and other chromosome 7 abnormalities are reported in 25% of cases.4 Diagnostic criteria as defined by the International JMML Working Group include peripheral blood monocytosis >1000/mm3, absence of the Philadelphia chromosome t(9:22) and the BCR-ABL1 fusion gene rearrangement, and bone marrow blasts |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|