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Acute lymphobl a stic leukemia (ALL)

Acute lymphobl a stic leukemia (ALL). Clonal proliferation and accumulation of blast cells in blood, bone marrow and other organs Disorder originates in single B or T lymphocyte progenitor Heterogenous disease with different biological subtypes I ncidence in adults : 20% of acute leukemias

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Acute lymphobl a stic leukemia (ALL)

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  1. Acute lymphoblastic leukemia (ALL) Clonal proliferation and accumulation of blast cells in blood, bone marrow and other organs Disorder originates in single B or T lymphocyte progenitor Heterogenous disease with different biological subtypes Incidence in adults : 20% of acute leukemias Etiology - unknown

  2. Acute leukemias - clinical features 1. Bleeding 2. Fever/infection 3. Bone/joint pain 4. Hepatomegaly 5. Splenomegaly 6. Lymphadenopathy 7. CNS involvement

  3. Acute leukemias - laboratory findings (1) 1. Blood examination - anemia, - thrombocytopenia, - variable leukocyte count, usually increased, - blood morphology: presence of blast cells 2. Bone marrow morphology - presence of blast cells, - suppression of normal hematopoiesis

  4. Acute leukemias - Laboratory findings (2) 3. Cytochemical stains 4. Immunophenotyping 5. Cytogenetics 6. Molecular studies

  5. Morphologic subtypes of acute lymphoblastic leukemias (FAB classification) Subtype Morphology Occurrence (%) L1 Small round blasts 75 clumped chromatin L2 Pleomorphic larger blasts20 clefted nuclei, fine chromatin L3 Large blasts, nucleoli,5 vacuolated cytoplasm

  6. Acute lymphoblastic leukemias - reactivity with special stains Subtype Peroxidase or Non-specific Periodic Sudan black esterase acid-Schiff L1 - - +++ L2 - - +++ L3 - - +++

  7. Immunologic classification of acute lymphoblastic leukemias B- lineage (80%) Markers Pro-B CD19(+),Tdt(+),CD10(-),CyIg(-), Common CD19(+),Tdt(+),CD10(+),CyIg(-), Pre-B CD19(+),Tdt(+),CD10(+),CyIg(+),SmIg(-) Mature-B CD19(+),Tdt(+),CD10(±),CyIg(±),SmIg(+) T-lineage (20%) Pre-T CD7(+), CD2(-), Tdt(+), Mature-T CD7(+), CD2(+), Tdt(+),

  8. Chromosomal/molecular abnormalities with prognostic significance in ALL Better prognosis - normal koryotype - hyperdiploidy Poor prognosis - t (8; 14) - t (4; 11) Very poor prognosis - t (9; 22); BCR/ABL (+)

  9. Risk classification in ALL 1. Standard risk 2. High risk 3. Very high risk

  10. High-risk ALL 1. Pre - T 2. Pro - B 3. Age > 35 years, 4. WBC > 30 G/L in B-ALL > 100 G/L in T-ALL 5. No remission after 4 weeks of induction therapy

  11. Very high-risk ALL Chromosome Philadelphia - positive or BCR/ABL (+)

  12. Treatment strategy in ALL

  13. In ALL the choice of treatment-strategy depends on: 1. Risk qualification 2. Immunophenotype of leukemic cells - T lineage, - early B lineage, - mature B lineage, 3.Age and biological condition 4. Goal of treatment

  14. Remission induction therapy in ALL 1. Antineoplastic treatment a/Drugs: prednisone, vincristine, asparginase, cyclophosphamide duanorubicin/adriablastin/epirubicin, cytosine arabinoside, b/Treatment duration: 4-8 weeks c/ No of courses: 1- 2 2. CNS prophylaxis 3. Supportive care 4. Treatment of complications

  15. Post-remission therapy in standard-risk ALL 1. Chemotherapy a/. Maintenance therapy: 6-mercaptopurine, methotrexate - for 2-3 years. b/. Intensification treatment periodically repeated: daunorubicin/adriablastin, prednisone, vincristine, cyclophosphamide. 2. CNS prophylaxis

  16. Post-remission therapy in high-risk ALL 1. Intensification treatment: amsacrine, mitoxantrone, idarubicine, high dose cytosine arabinoside, high dose methotrexate, high dose cyclophosphamide. 2.Hematopoietic stem cell transplantation - high-dose therapy - reduced intencity conditioning

  17. Post-remission therapy in very high-risk ALL - High-dose therapy( reduced-intencity ?) + allogeneic stem cell transplantation

  18. Treatment results in ALL • Adults • Complete remission (CR) 80-85% • Leukemia-free survival (LFS) 30-40% • Children • Complete remission (CR) 95-99% • Leukemia-free survival (LFS) 70-80%

  19. AutoHSCT in ALL • Treatment related mortality (TRM) 2-8% • CR1 • LFS 42% (15-65%) • RI ( relapse incidence) 51% • CR2 • LFS 24% (20-25%) • RI 60%

  20. AlloHSCT in ALL • Sibling donor CR1 >CR2 relapsed/refractory LFS 51% (21-80) 34% (13-42) 20% (12-33) RI 26% (9-50) 47% (40-69) 71% (59-76) TRM 29% (12-42) • Matched unrelated donor LFS 39% (38-42) RI 22% (19-23) TRM 48%

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