Myeloproliferative Disorders Flashcards
Basophilia means
CML
What does basophilia mean
CML
Basophilia means what
CML
Why is there basophilia in CML
CML results in proliferation of all myeloid lineage cells
including basophils
this is the only real cause we know of that causes basophilia
Myeloproliferative disorders may lead to what complications
hyperuricemia and gout
marrow fibrosis
CML translocation
t(9;22) Philadelphia chromosome
*also seen in bad form of B-ALL
In which cell in the myeloid lineage is the mutation driving CML
in hematopoietic stem cell
CML may progress to
AML or ALL
*may progress to either because driving mutation is in the hematopoietic stem cell and not a more mature myeloid progenitor cell
t(9;22)
pathology
disease
treatment
BCR-ABL fusion: constant activation of tyrosine kinase
CML and some B-ALL
imatinib is Tx, inhibits TK activity
CML stages
chronic: steady
accelerated: on its way to acute
transformation: acute leukemia, either AML or ALL
CML moving into accelerated or acute
may be marked by
hepatosplenomegaly
Leukocyte alkaline phosphatase (LAP) stain
usefulness in CML
CML are LAP -
LAP commonly used in leukemoid reaction (rxn to infection) to help destroy infection
CML labs:
LAP stain
WBC w/ diff
cytogenetics
LAP negative
basophilia
t(9;22): Ph+, BCR-ABL TK mutation
Polycythemia vera
driving mutation
JAK2 kinase mutation
Polycythemia vera
presentation
*hyperviscocity Sx blurry vision and headache venous thrombosis, ie Budd-Chiari flushed face itching, especially after bathing
Why is there itching after bathing in polycythemia vera
release of histamine from mast cells
Tx of polycythemia vera
phelbotomy
hydroxyurea (2nd line)
Polycythemia
EPO
SaO2
low EPO
normal SaO2
Reactive polycythemia to low O2
EPO
SaO2
high EPO
low SaO2
Ectopic EPO production
cancer
EPO
SaO2
renal cell carcinoma
high EPO
normal SaO2
Driving mutation in essential thrombocythemia
JAK2 kinase mutation
Sx of essential thrombocythemia
bleeding and/or thombosis
platelets either hyperfunction of not at all really
Which myeloproliferative disorder does not have an increased risk for gout
essential thrombocythemia
because platelets are derived from megakaryocytes
don’t have their own nuclei
Myelofibrosis caused by proliferation of
megakaryocytes
PDGF (platelet-derived growth factor) release
