@inproceedings{01GWP8V9AE2NPK4AWCQBRWN7X4,
  abstract     = {{Introduction:
Juvenile myelomonocytic leukemia (JMML) and RAS-associated lymphoproliferative disease (RALD) are both hematologic diseases with related gene mutations, such as KRAS or NRAS. In addition, most of the RALD patients fulfill the diagnostic criteria of JMML making it a real challenge to discriminate between both diseases. However, the prognosis of JMML patients is generally poor, whereas the long-term follow-up of patients with RALD suggests a more indolent course. The objective of this study was to establish clinical and biological hallmarks that could differentiate JMML from RALD in patients with KRAS and NRAS mutations, to optimize patient care. 
Methodology:
We conducted a systematic review with data about the clinical and biological characteristics collected from case reports and cohort studies describing patients diagnosed with KRAS- or NRAS-mutated JMML or RALD. Statistical analyses were performed with SPSS. To assess the differences between patients diagnosed with JMML and patients diagnosed with RALD, the Chi-Square test, and Fisher’s Exact test were used to compare qualitative variables. To compare quantitative variables, an Independent two-sample t-test and Mann-Whitney U test were used.
Results:
Comparing the categorical variables, significant differences in the gender distribution, the presence of an abnormal karyotype, monocytosis, and granulocyte-macrophage colony-stimulating factor (GM-CSF) hypersensitivity were noted between both patient groups. When comparing the blood cell counts, a higher white blood cell (WBC) count, monocyte count, lymphocyte count, and percentage of circulating blasts in peripheral blood (PB) were reported in the JMML group. When comparing KRAS-mutated JMML and KRAS-mutated RALD, differences in the gender distribution, the presence of an abnormal karyotype, and age at diagnosis/onset were observed. When comparing the blood cell counts, a higher WBC count and monocyte count were found in the JMML group. When comparing NRAS-mutated JMML and NRAS-mutated RALD, a difference in the distribution of splenomegaly was seen, together with the higher WBC count in the JMML group. Interestingly, a diagnosis of RALD is more likely if autoimmune manifestations are present.
Conclusion:
With this study, we found a clear overlap between both diseases, although some differences could be noted. Given this overlap, JMML and RALD may be two extremities of the same disease spectrum rather than two distinct entities.}},
  author       = {{Verhulst, Hanne and Van Camp, Laurens and Hofmans, Mattias and De Moerloose, Barbara}},
  booktitle    = {{BVK-SBP Congress 2023, Abstracts}},
  language     = {{eng}},
  location     = {{The Egg, Brussels, Belgium}},
  title        = {{The clinical spectrum and biological findings in RAS-associated juvenile myelomonocytic leukemia and RAS-associated autoimmune leukoproliferative disorder}},
  year         = {{2023}},
}