B-Cell Acute Lymphoblastic Leukemia/Lymphoblastic Lymphoma
Clinical
- Primarily a disease of childhood, 75% <6 yo
- Increased risk in trisomy 21
- LBL has predominantly extramedullary disease with limited BM involvement (<25%)
- Most common sites of LBL involvement are skin, soft tissue, bone, and LN
- Presents with bone pain, cytopenias, LAD
Risk stratification:
- Traditionally based age, WBC count and response to chemotherapy; now often based on genetics
Good Prognosis:
- Children
- ETV6-RUNX1 - very good prognosis
- Hyperdiploidy
Bad Prognosis:
- Infants and older adults
- Patients with higher WBC count, CNS involvement, MRD, etc
- t(9;22)- worst prognosis
- KMT2A
- Hypodiploidy
- iAMP21
- BCR-ABL-like
Intermediate/unknown:
- t(1;19)
- t(5;14)
Dr. Laura Brown @lauraebrown
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