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MMSC433 Exam 3 Objectives Questions with 100% Verified
Answers UPDATED!!!
Myeloproliferative disorders
CML → Chronic Myeloid Leukemia
PV → Polycythemia Vera
ET → Essential Thrombocythemia
PMF → Primary Myelofibrosis
Others:
-Chronic Neutrophilic Leukemia
-Chronic Myelomonocytic Leukemia
-Juvenile Myelomonocytic Leukemia
CML
Single genetic translocation in pluripotential hematopoietic
stem cell producing a clonal overproduction of the myeloid cell
line, resulting in a preponderance of increased immature cells in
the neutrophilic line
-chronic phase can turn into accelerated phase in 3-4 yrs if
untreated
-can transition to AML or ALL
CML clinical features
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Anemia
Bleeding
Splenomegaly
Infections w strep pneumoniae
CML chromosomal abnormality
Philadelphia chromosome
Philadelphia chromosome
translocation of chromosome 9 to BCR of band q11 of
chromosome 22
Normal ABL (abselson) gene
functions as kinase (adds phosphate)
normal BCR gene
functions as GTPase activating protein (GAP)
-active when bound to GTP
BCR-ABL1 fusion gene
identified w/ philadelphia chromosome (positive ALL)
CML morphology/lab findings
-RBC ↓
-LAP ↓
-Reticulocytes normal
-lymphocytes/PLTs normal
-WBC, neutrophil, basophil, eosinophil, myelocyte ↑
-Gaucher-like cells (sea blue)
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-Green-gray crystal ↑
-All extramedullary tissue present
CML Treatment
-BM + stem cell transplant (CURE)
-Imatinib mesylate (Gleevec) → treatment of choice
Goal of CML therapy
absence of Ph1, BCR/ABL
State WHO criteria for the accelerated phase of CML
-PB leukocytosis due to increased number of neutrophils and
their precursors
-Dysgranulopoiesis - may include abnormal chromatin clumping
-No or minimal absolute basophilia; basophils usually <2% of
leukocytes
-No or menial absolute monocytosis; monocytes <10% of
leukocytes
-Hypercellular BM with granulocytic proliferation and
granulocytic dysplasia, with or without dysplasia in the
erythroid and megakaryocytic lineages
-<20% blasts in the blood and BM
-No evidence of PDGFRA, PDGFRB, or FGFR1 rearrangement, or
PCM1-JAK2
-Not meeting WHO criteria for BCR-ABL+ CML, PMF, PV, or ET
PV
MMSC433 Exam 3 Objectives Questions with 100% Verified
Answers UPDATED!!!
Myeloproliferative disorders
CML → Chronic Myeloid Leukemia
PV → Polycythemia Vera
ET → Essential Thrombocythemia
PMF → Primary Myelofibrosis
Others:
-Chronic Neutrophilic Leukemia
-Chronic Myelomonocytic Leukemia
-Juvenile Myelomonocytic Leukemia
CML
Single genetic translocation in pluripotential hematopoietic
stem cell producing a clonal overproduction of the myeloid cell
line, resulting in a preponderance of increased immature cells in
the neutrophilic line
-chronic phase can turn into accelerated phase in 3-4 yrs if
untreated
-can transition to AML or ALL
CML clinical features
,2|Page
Anemia
Bleeding
Splenomegaly
Infections w strep pneumoniae
CML chromosomal abnormality
Philadelphia chromosome
Philadelphia chromosome
translocation of chromosome 9 to BCR of band q11 of
chromosome 22
Normal ABL (abselson) gene
functions as kinase (adds phosphate)
normal BCR gene
functions as GTPase activating protein (GAP)
-active when bound to GTP
BCR-ABL1 fusion gene
identified w/ philadelphia chromosome (positive ALL)
CML morphology/lab findings
-RBC ↓
-LAP ↓
-Reticulocytes normal
-lymphocytes/PLTs normal
-WBC, neutrophil, basophil, eosinophil, myelocyte ↑
-Gaucher-like cells (sea blue)
, 3|Page
-Green-gray crystal ↑
-All extramedullary tissue present
CML Treatment
-BM + stem cell transplant (CURE)
-Imatinib mesylate (Gleevec) → treatment of choice
Goal of CML therapy
absence of Ph1, BCR/ABL
State WHO criteria for the accelerated phase of CML
-PB leukocytosis due to increased number of neutrophils and
their precursors
-Dysgranulopoiesis - may include abnormal chromatin clumping
-No or minimal absolute basophilia; basophils usually <2% of
leukocytes
-No or menial absolute monocytosis; monocytes <10% of
leukocytes
-Hypercellular BM with granulocytic proliferation and
granulocytic dysplasia, with or without dysplasia in the
erythroid and megakaryocytic lineages
-<20% blasts in the blood and BM
-No evidence of PDGFRA, PDGFRB, or FGFR1 rearrangement, or
PCM1-JAK2
-Not meeting WHO criteria for BCR-ABL+ CML, PMF, PV, or ET
PV
