B cell acute lymphoblastic leukemia/lymphoma
B cell acute lymphoblastic leukemia/lymphoma
Updated: 08/28/2024
© Jun Wang, MD, PhD
General features
- Most common pediatric leukemia
- Lymphoma is defined as localized disease with <20% lymphoblasts in marrow and peripheral blood
Clinical presentations
- Abrupt stormy onset
- Pancytopenia due to bone marrow depression
- Bone symptoms: pain, tenderness
- Lymphadenopathy
- Internal organ involvement: hepatosplenomegaly, testicular involvement, central nervous system manifestations
Key Laboratory findings
- Lymphoblast in peripheral blood and/or bone marrow
Key morphological features of lymphoblast
- Larger than normal lymphocytes
- Scant cytoplasm
- NO granules
- NO Auer rods
Markers
- CD34, CD99, TdT, bcl-2, CD19, CD79a, PAX5
Genetic abnormalities
- Various
- Hyperdipoidy (>50 chromosomes): Excellent prognosis with antimetabolite-based therapy
- Hypodiploidy (< 44 chromosomes): Poor prognosis, high frequency of RAS pathway mutation
- t(12;21)(p13;q22): ETV6-RUNX1
- t(9;22)(q34;q11.2): bcl-abl1, prognosis improved with imatinib addition to intensive chemo
- t(4;11)(q21;q23): MLL-AFF (AF4)
- Translocation involving cytokine receptor-like factor 2 (CRLF2): poor prognosis
- JAK2 mutation (R683G: NOT as V617F seen in MPN): poor prognosis
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ReplyDeletewhy not proper treatment for acute lymphoblastic leukemia
acute lymphoblastic leukemia
This blog is designed to help medical students learning basic pathology in their preclinical years, and for their preparation of COMLEX 1 and/or USMLE step 1. Treatment is barely needed, unless it is associated with specific pathogenesis, such as all-tran-retinoic acid for APL.
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