Hematopoietic Stem Cell Transplantation (HSCT)
Farnesyl Protein Transferase Inhibitor
Incidence: 1.2 per million children per year
Median age at diagnosis: 1.8 years; 35% below 1 year of age, and only 4% above 5 years of age
Male : female ratio of 2:1
Association with neurofibromatosis type 1 (NF-1): Children with NF-1 have more than a 200-fold increased risk of JMML. Fifteen percent of children with JMML have NF-1.
Children with Noonan syndrome or trisomy 8 mosaicism are at increased risk of developing JMML. Most cases of neonates with Noonan syndrome with JMML-like presentation resolve spontaneously.
JMML usually occurs before 2 years of age. Physical findings include:
Skin : eczema, xanthoma, café-au-lait spots, macular-papular rash
Respiratory symptoms: chromic tachypnea, cough, wheezing
- PHILIP LANZKOWSKY. MANVAL of pediatric Hematology and oncology (Fourth Edition).
ELSEVIER ACADEMIC PRESS
- Philip Lanzkowsky. MANVAL OF Hediatric Hematology AND Oncology (SEOND EDITON).
- NELSON TEXTBOOK OF PEDIRICS (17th EDITON). SAVNDERS