Acute Lymphoblastic Leukemias/Lymphomas ALLs

Type
1. Precursor B (pre-B) ALL
  1. Childhood
    1. Peaks age of 3
      1. Bone marrow pre-B cell great
  2. Acute "Leukemias"
2. Precursor T (pre-T) ALL
  1. Adolescent males
    1. Age when thymus is maximal size
  2. Thymic "Lymphomas"
Morphology
1. Marrow is Hypercellular
2. Macrophages ingesting apoptotic tumor cells
  1. "Starry Sky" appearence
3. Similar morphology AML
  1. Lymphoblasts are MPO Negative and PAS Positive
Immunophenotype
1. 95% TdT Positive
2. pre-B ALL >> CD19
3. pre-T ALL >> CD1a
Molecular Pathogenesis
1. Hyperploidy (>50 chromosomes)
2. NOTCH1 >> 70%T-ALLs
3. PAX5 >> B-ALLs
Clinical Features
1. AML is clinically very similar
2. Accumulation of neoplastic "blasts" in bone marrow
  1. Supresses normal hematopoiesis by physical crowding ...
3. Abrupt stormy onset
4. Depression of marrow function
5. Bone pain
6. CNS manifestations
Prognosis
1. with aggressive chemotherapy
  1. 95% of children with complete remission
    1. 75-85%are cured
  2. only 35-40% of adults are cured
2. Causes of Bad Prognosis
  1. age under 2
    1. strong association with MLL gene translocations
  2. presentation in adolescence
  3. Presence of Philadelphia chromosome, t(9;22)

Resource summary

	Created by Dr. Ömer Topcu about 10 years ago