Term
Acute myeloid leukemia (AML)
-mechanism
-symptoms
-tests, findings
-treatment
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Definition
Mechanism:
- >20% blasts in BM causing hematopoeitic cell suppression
Symptoms:
- anemia
- neutropenia
- thrombocytopenia
Tests:
- Auer rods, except M0
- CD34+
- CD13, 15, 33
- cytogenetics, FISH, RT-PCE = M3 APL
Treatment:
- anthracycline, cytarabine (induction tx) until bone marrow aplasia (5% blasts)
- if less favorable patient:
~ high dose chemo followed by autologous stem cell transplant
- Exception: M3 APL
~ treat with ATRA +/- arsenic trioxide
~ fresh frozen plasma (DIC) |
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Term
M0: undifferentiated AML
-Key findings
-Prognosis |
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Definition
Key findings:
- myeloperoxidase (-)
- no auer rods
- >20% blasts in BM; some in peripheral smear
Prognosis:
- poor |
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Term
M1 AML
- Key findings
- Prognosis |
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Definition
Key findings:
- >90% myeloblasts
- peroxidase (+)
- few granules or auer rods
- t(9;22) in 10-15% cases
Prognosis:
- aggressive |
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Term
M2 AML
- Key findings
- Prognosis |
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Definition
Key findings:
- >20% blasts
- some maturation of granulocytes
- Auer rods common
- t(8;21) in 60-65% of cases
Prognosis:
- good prognosis with t(8;21) |
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Term
M3: acute promyelocytic leukemia (APL)
- Key findings
- Treatment |
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Definition
Key findings:
- >20% promyelocytes
- Auer rod bundles (faggot cells)
- DIC is common (consumptive coagulopathy)
- t(15;17)
Treatment:
- All-trans retinoic acid (ATRA) +/-
- Arsenic trioxide |
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Term
M4: acute myelomonocytic leukemia
- Key findings |
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Definition
Key findings:
- 20% myeloblasts
- 20% monoblasts
- M4eo (inv16) variant is common |
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Term
M5: acute monoblastic leukemia
- Key findings
- Prognosis |
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Definition
Key findings:
- >20% monoblasts, <20% myeloblasts
- high WBC count
- consumptive coagulopathy (DIC)
- Subtypes with CNS (meningeal) involvement → HA, diplopia
- swollen gums
- 11q23 common
- Flt3 gene in subtypes (CNS)
Prognosis:
- poor |
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Term
M6: erythroleukemia
- Key findings
- prognosis
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Definition
Key findings:
- >50% erythroblasts in BM
- >20% nonerythroid cells in BM are myeloblasts
- peripheral blood may have NRBCs and blasts may be found
- M:E greatly reduced
- ineffective erythropoiesis
- chromosome 5 &/or 7 abnormalities
- present with profound anemia
Prognosis:
- aggressive clinical course; hard to treat |
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Term
M7: acute megakaryoblastic leukemia
- Key findings |
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Definition
Key findings:
- >50% megakaryoblasts predominate in BM
- rare
- auer rods absent
- bone marrow fibrosis and increased marrow reticulum often seen
- ↑ in Down's syndrome age < 3 y/o
- Patients present with bone marrow failure (pancytopenia) and organomegaly (in children)
Prognosis:
- aggressive clinical course |
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Term
Myelodysplastic syndrome:
- Mechanism
- Symptoms
- Cell types
- Genetics
- Treatment
- Prognosis |
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Definition
Mechanism:
- maturational defects causing ineffective hematopoeisis and peripheral blood cytopenias
- <20% blasts in BM
Symptoms:
- pancytopenia results in:
~anemia
~hemorrhages
~neutropenia→ infection
Genetics:
- chromosome 5 &/or 7 abnormalities
Treatment:
- allogeneic stem cell transplant
- Transfusions of RBCs
- Antibiotics
- Colony stimulating factor (EPO, G-CSF)
- azacytidine + decitabine → interference of hypermethylation of DNA causing regulatory genes reactivation; new treatments
- Lenalidomide: patients with 5q deletion, $$$
Prognosis:
- slowly progressive diseases
- ~30% → AML (difficult to treat) |
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Term
| WHO classification of MDS |
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Definition
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