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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