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31 Cards in this Set

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What are the 4 chronic myeloproliferative disorders?
1.) Chronic myelogenous leukemia (CML)
2.) Polycythemia vera
3.) Idiopathic myelofibrosis
4.) Essential thrombocythemia
What is the definition of chronic myeloproliferative disorders (MPDs)?
- interrelated clonal abnormalities
- disorder of hematopoiesis with an excess of phenotypically normal mature cells, mostly myeloid
- one type my evolve to another or acute
What is chronic myelogenous leukemia (CML)?
a clonal myeloproliferative expansion of transformed, primitive hematopoietic progenitor cells
What lines are involed in CML?
1.) myeloid
2.) monocytic
3.) erythroid
4.) megakaryocytic
5.) B-lymphoid
6.) some T-lymphoid cells
Age range of CML
30-50 years old
Pathophysiology of CML
- disorderly expansion of myeloid progenitor cells results from alterations in their proliferative capacity and a shift in the balance between self-renewal and differentiation toward differentiation
- increased progenitor cells
- decreased pool of stem cells
Cytogenics of CML
- Ph1 chromosome result from reciprocal translocation of DNA between chromosomes 9 and 22
- makes 2 novel fusion genes: BCR:ABL on 22q-(Philadelphia) and ABL-BCR on 9q+
ABL gene
- produces a protein tyrosine kinase
- fusion protein BCR-ABL has constitutive kinase activity that deregulates signal transduction pathways causing:
* abnormal cell cycling
* inhibition of apoptosis
* increased proliferation of cells
Phases of CML
1.) Initial (chronic)
2.) Accelerated
3.) Blast crisis (acute)
Initial Phase
- chronic phase
- 85% cases diagnosed
- prognosis 3-5 years
Accelerated Phase
- blasts >10%
- basos and eos >20%
- WBC >50x10^9/L
- Hct <25%
- plt <100x10^9/L
- Pelger-Huet, nucleated RBC, megakaryocytic nuclear fragments
Blast Crisis
- acute phase
- 3/4 of patients enter a gradual transformation to a blast crisis
Leukocyte alkaline phosphatase (LAP)
- used to differentiate between CML and a leukemoid reaction (severe infection or inflammation)
- CML: LAP decreased
- leukemoid reaction: LAP increased
- during remission, the LAP may return to normal
Clinical use of detecting gene rearrangements involving the BCR and c-abl genes
1.) Confirmation of Ph1-positive cases of CML
2.) Diagnosis of Ph1-negative cases of CML
3.) Diagnosis of CML presenting in blast crisis
4.) Monitoring of patients with CML during and after therapy for detection of minimal residual disease
5.) Confirmation of remission
6.) Early detection of relapse
Diagnostic procedures
1.) PCR
2.) Southern blot of DNA break points
3.) Northern blot to detect BCR-ABL RNA transcripts
4.) Wester blot-immunoprecipitation of antibodies against the N-term of BCR and C-term of ABL
CML treatment
- imatinib: a selective inhibitor of the BCR-ABL tyrosine kinase, which occupies the ATP-binding site of tyrosine kinase
* prevents phosphorylation of substrates that are involved in regulating cell cycle
* for ones w/o donor and can't do transplantation
* can add IFN-alpha, cytarabine, hydroxyurea, or arsenic trioxide
* other: ABL kinase and signal transduction inhibitors, enhancing Acr-Abl degradation, proteasome inhibition, and immune-based approaches
What are the 4 chronic myeloproliferative disorders?
1.) Chronic myelogenous leukemia (CML)
2.) Polycythemia vera
3.) Idiopathic myelofibrosis
4.) Essential thrombocythemia
What is the definition of chronic myeloproliferative disorders (MPDs)?
- interrelated clonal abnormalities
- disorder of hematopoiesis with an excess of phenotypically normal mature cells, mostly myeloid
- one type my evolve to another or acute
What is chronic myelogenous leukemia (CML)?
a clonal myeloproliferative expansion of transformed, primitive hematopoietic progenitor cells
What lines are involed in CML?
1.) myeloid
2.) monocytic
3.) erythroid
4.) megakaryocytic
5.) B-lymphoid
6.) some T-lymphoid cells
Age range of CML
30-50 years old
Pathophysiology of CML
- disorderly expansion of myeloid progenitor cells results from alterations in their proliferative capacity and a shift in the balance between self-renewal and differentiation toward differentiation
- increased progenitor cells
- decreased pool of stem cells
Cytogenics of CML
- Ph1 chromosome result from reciprocal translocation of DNA between chromosomes 9 and 22
- makes 2 novel fusion genes: BCR:ABL on 22q-(Philadelphia) and ABL-BCR on 9q+
ABL gene
- produces a protein tyrosine kinase
- fusion protein BCR-ABL has constitutive kinase activity that deregulates signal transduction pathways causing:
* abnormal cell cyclin
* inhibition of apoptosis
* increased proliferation of cells
Phases of CML
1.) Initial (chronic)
2.) Accelerated
3.) Blast crisis (acute)
Initial Phase
- chronic phase
- 85% cases diagnosed
- prognosis 3-5 years
Accelerated Phase
- blasts >10%
- basos and eos >20%
- WBC >50x10^9/L
- Hct <25%
- plt <100x10^9/L
- Pelger-Huet, nucleated RBC, megakaryocytic nuclear fragments
Blast Crisis
- acute phase
- 3/4 of patients enter a gradual transformation to a blast crisis
Leukocyte alkaline phosphatase (LAP)
- used to differentiate between CML and a leukemoid reaction (severe infection or inflammation)
- CML: LAP decreased
- leukemoid reaction: LAP increased
- during remission, the LAP may return to normal
Clinical use of detecting gene rearrangements involving the BCR and c-abl genes
1.) Confirmation of Ph1-positive cases of CML
2.) Diagnosis of Ph1-negative cases of CML
3.) Diagnosis of CML presenting in blast crisis
4.) Monitoring of patients with CML during and after therapy for detection of minimal residual disease
5.) Confirmation of remission
6.) Early detection of relapse
Diagnostic procedures
1.) PCR
2.) Southern blot of DNA break points
3.) Northern blot to detect BCR-ABL RNA transcripts
4.) Wester blot-immunoprecipitation of antibodies against the N-term of BCR and C-term of ABL