HEME / LYMPH MSII
Terms
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- Auer rods & granules indicated what?
-
AML (Acute Myeloid Leukemia) or myelodysplasia
Auer rods are typical of blast cells - Smudge cells are indicitive of _____?
- CLL
- Name the myeloproliferative diseases
-
P. Vera
ET (essential thrombocythemia)
CML (chronic myelogenous leukemia
Chronic Idiopathic myelofibrosis -
Philadelphia Chromosome
(what is it) -
t(9:22)
fusion of BCR/ABL genes, which results in enhanced Tyrosine Kinase activity -
Philadelphia Chromosome
(what's it indicitive of) -
All CML: with 210-kDa protein
Some ALL: 190 or 210 kDa
Chronic Neutropilic Leukemia: 230-kDa protein & lower WBC & Slower progression - diagnosis of CML
-
hx & pe
blood smear & bm biopsy (WBCs of various diff, blasts < 5%)
cytogenetics (Phila chrom);
PCR (looking for BCR-ABL gene)
LAP (low) - Hydroxyurea does what?
- decreases marrow proliferation; causes a hematologic response, but remission is rare
- Tyrosine Kinase is responsible for what?
- Stimulates the growth cycle (mitosis, etc...).
- Tx of CML
-
Hydroxyurea
Interferon
ST1571 (Gleevec, Imatinib) - Interferon does what?
-
is anti-proliferative; it restores cell adhesion to marrow stroma, and decreases Philadelphia chromez by 35%!
Complete hematologic response (30~68%)! - How does ST1571 work (Gleevec, Imatinib)?
- Inhibits tyrosine kinase, decreasing mitosis signals & promoting apoptosis
- CD5+ CD19+ CD23+
- CLL
- CD5+ CD19+ CD23-
- Mantle Cell lymphoma
- CLL Staging Criteria (0-IV)
-
0: lymphocytosis in PB & BM
I: 0 + lymphadenopath
II: + spleen & liver
III: + anemia
IV: thrombocytopenia - CD5- CD23- CD25+ CD103+
- Hairy Cell Leukemia
- Tx for Hairy Cell Leukemia
-
2-CDA (7 days = 90% complete response)
... also in-alpha, pentostatin, or splenectomy