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Clinical Leukemia - Dr. Raphael

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What are some etiologies of leukemia? (5)
1) chemical exposure (benzene)
2) radiation therapy
3) chemo therapy*
4) inherited genetic instability (Down's)
5) transforming virus
* major cause nowadays
AML cytogenetics ~
M2 = t(___:___)
M3 = t(___:___)
M4 = inversion ___
Biphenotypic AML = t(___:___)
8:21
15:17
16
4:11
How does AML present clinically?
1) DIC
2) leukostasis (>75000 blasts)
3) hyperuricemia
4) hepatosplenomegaly
5) pancytopenia
6) "tumor lysis syndrome"
What are some good cytogenetic prognostic factors for AML?
inversion 16, t(15;17), t(8;21)
What are some bad prognostic factors for AML?
(age, WBC count, etc.)
> 60 yrs
high WBC count
prior Myelodysplastic Syndrome
prior chemo therapy
What is consolidation therapy for AML?
HIGH dose cytarabine
(tox: leukopenia, thrombocytopenia, megaloblastic anemia)
What is used in targeted therapy for AML?
anti-CD33, because young cells have CD33
Acute Promyelocytic Leukemia (M3 AML) responds to _____ & _____ therapy.
all trans retinoic acid
arsenic
What stain can differentiate between lymphoid blasts and myeloblasts?
PAS - lymphoid blasts have glycogen
Pre-B lymphoblsts are positive for _____ & _____.
TDT & CD10 (CALLA)
ALL affects mostly _____.
children (30% of childhood leukemias)
ALL has higher incidence of tumor spread to _____ & _____.
CNS, testes
Treatment of ALL includes:
induction
consolidation
maintenance
CNS prophylaxis (intrathecal)
allogeneic transplant
Cytogenetic abnormality in CML:
t(9:22) --> bcr-abl chimeric protein --> super pluripotent stem cell --> become either lymphoid or myeloid cells
CML clinical features:
1) no sxs in early stage, just high WBC
2) rising WBC in all stages
3) low neutrophilic ALP
4) accelerated phase - anemia, thrombocytopenia, but basophilia and fibrosis
CLL expresses which surface protein marker?
CD5
CLL typically occurs in which gender and age population?
Men > 50 yrs
Clinicaly features of CLL:
1) hypogammaglobulinemia
2) inc. infections
3) T-cell dysregulation
4) second malignancy
5) autoimmunity (hemolytic anemia)
Indications for initiating therapy in CLL:
massive LNs and spleen
CLL Tx:
1) alkylater therapy (chlorambucil, cytoxan)
2) purine analog (fludarabine)
3) rituximab, campath
MDS definition:
Absence of pluripotent progenitors.
MDS presentation:
inc. incidence with age, progressive pancytopenia, macrocytosis, poikilocytosis, anisocytosis

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