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28 Cards in this Set

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CML
Cancer of the white blood cells
Malignant cell is relatively immature stem cell
Result is excess production of mature cells of multiple lineages
CBC for CML
Elevated total white cell count
Differential reveals elevated numbers of neutrophils, bands, myelocytes, metamyelocytes, eosinophils, basophils, platelets
Diagnosing CML
Find the Philadelphia chromosome
t(9;22)
Chromosome 9: abl gene (Abelson leukemia virus)
Chromosome 22: bcr gene (breakpoint cluster region)
abl protein
The abl protein is a tyrosine kinase, which is an enzyme involved in signal transduction
When the t(9;22) is present, the tyrosine kinase is always phosphorylated (ie always “on”)
This provides a constant signal to certain pathways that results in cell growth that exceeds apoptosis
chromosome 22 and bcr gene
Chromosome 22 can break in different regions within the bcr gene, resulting in different sizes of bcr-abl protein products:
p190 (190 kDa protein): seen in ALL
p210 (210 kDa protein): seen in CML
Different phases of CML
Chronic phase: <5% blasts in the marrow
Accelerated phase:
Many different criteria
5-20% blasts in the marrow
Blast crisis:
>20% blasts in the marrow
Just like any other acute leukemia
Can be myeloid or lymphoid (flow cytometry)
Treatment of CML past and present
Pre-Gleevec era:
High dose interferon
Very toxic: fever, flu-like symptoms, fatigue
Allogeneic stem cell transplant
Very high associated morbidity and mortality

Gleevec (imatinib mesylate)
TKI (tyrosine kinase inhibitor)
STI (signal transduction inhibitor)
Small molecule inhibitor
Pill taken once a day
Side effects:
Mild nausea and vomiting
Periorbital edema
Pleural effusions
Well-tolerated
Gleevec
Binds to c-kit
On GISTs
(GI stromal tumors)
Binds to PDGFR-alpha
Seen in hypereosinophilic syndrome
FDA approved in 2001 for CML
No real long term follow-up
Gleevec resistance is developing
Some via T315I mutations
Second generation Tyrosine Kinase inhibitors
Dasatinib
Nilotinib
Neither are effective against the T315I mutation
treament of accelerated phase
Gleevec
Blast crisis
treated like the acute leukemia
Myeloid blast crisis: “7+3” cytarabine x 7 days with daunorubicin x 3 days
Lymphoid blast crisis: Complex multi-agent chemotherapy regimen; exactly what we would use for de novo ALL
CLL
Cancer of the white blood cells
Malignant cell is more differentiated than CML
Results in excess numbers of mature-appearing lymphocytes
Continuum with SLL (small lymphocytic lymphoma) that has no circulating neoplastic cells and resides in lymph nodes
CBC for CLL
Elevated total white cell count
Differential is primarily lymphocytic
Hemoglobin and platelets are normal
Except in advanced stages of disease when Hb and platelets can be low
Diagnosis of CLL
Peripheral blood sample for flow cytometry
Do not need a bone marrow for diagnosis
(+) CD5, CD19, CD23
(+/-) CD20 (weak expression)
(+) surface immunoglobulin
Light chain restriction
Only kappa or lambda, not both
Send chromosomal studies to get information on prognosis
steroids are only cytotoxic to
lymphoid cells
p210 kDa protein (bcr-abl)
indicative of CML
CLL stage 0
Lymphocytosis only
>10yr survival
low risk
CLL stage I
intermediate risk
Lymphocytosis
Lymphadenopathy
>8yr survival
CLL stage II
Lymohocytosis, splenomegaly, intermediate risk
~5yr survival
CLL stage III
high risk, lymphocytosis, Hb<11 due to progression of CLL in marrow
Survival 8-12 months
CLL sateg IV
lymphocytosis
plts<100K due to progression of CLL in marrow
high risk
8-12 month survival
CLL and immune dysregulation
Difficult to fight infection
Often need prolonged courses of antibiotics
Hypogammaglobulinemic
Quantitative immunoglobulins often reveal patients to be pan-hypoglobulinemic
If patients have persistent infections or infections severe enough to require hospitalization, will treat with IVIG
CLL and overactive immune system
Inappropriate destruction of “self” cells
Autoimmune hemolytic anemia
Immune thrombocytopenic purpura
Labs for AIHA are the same as with any other:
Elevated LDH, bilirubin
Low Hb, haptoglobin
Can be Coombs positive
Generally treat with steroids
Don’t need a bone marrow to make this diagnosis
Richter’s transformation
Development of diffuse large B cell lymphoma arising from one CLL clone
May have B symptoms, one area of lymphadenopathy out of proportion to others
PET scan will show transformed sites
CLL is not PET avid
DLBCL is very PET avid
Must document with biopsy to prove transformed disease
Treat with DLBCL regimen such as R-CHOP
CLL chemo is ineffective
Pts are still left with underlying CLL after treatment complete
Transformation to prolymphocytic leukemia
CLL
>55% prolymphocytes
Treatment is different from standard CLL treatment
CLL treatment
Chemotherapy
Purine analog based
Fludarabine based
Pentostatin based
Patients are at significant risk for tumor lysis syndrome with the first cycle of treatment
Hydration and frequent lab monitoring is important
Hairy Cell Leukemia
Comprises 2% of all leukemias
Very slow-growing
B cell malignancy (CD19, 20, 22)
Has aberrant expression of T cell marker CD103
TRAP-positive (tartrate-resistant acid phosphatase), a stain
Clinically, notable for very large spleens, and dry taps on bone marrows

In the bone marrow, cells look like fried eggs
Marrow also has lots of fibrosis
Treatment is also with purine analogs
Cladribine
Hairy cell leukemia treatment
clarabine- purine analog

so slow growing may only get one treatment in a lifetime