Myeloproliferative Diseases Flashcards
What are the two things that tie the myeloproliferative disorders together?
- ) Each affects multiple cell lineages
2. ) Each tends to end in an acute leukemia
Chronic Myelogenous leukemia: causes
We don’t know, radiation, benzene
CML: what is in Periph blood
myeloblast, progranulocyte, bands, segs…everything you would expect to see in the bone marow in the myeloid line.
What sets CML apart as far as peripheral blood smears go?
Basophilia….this is in nothing else
Other lab findings in CML
Uric acid (always appears when there is increased white cell count in blood), Alkaline phosphatase, B12
Most common chromosome abnormality?
t(9;22) philadelphia chromosome…BCR ABL ( end up with uncontrolled proliferation)
What is the terminal event in the disease?
Blast crisis- all myeloblasts
What is the most common modern therapy for CML?
Imatinib
How does Imatinib work?
Blocks the ATP receptor in the BCR-ABL tyrosine kinase
What are the the 3 myeloproliferative disorders characterized by Jak-2 mutations?
Primary Myelofibrosis, Polycythemia Vera, Thrombocythemia
What is Jak 2 associated with that makes it so important?
The EPO receptor. Signaling of EPOr starts with Jak2. It is attached to EPOr. Drives proliferation and differentiation of red cells.
What is Myelofibrosis
A disease where the bone marrow is filled up with fibrous tissue. Can no longer make bone marrow there so it begins to make bone marrow in the liver, spleen, and other areas. Mostly type 3 collagen deposited. This is a result of cytokines released frommegakaryocytes.
What is the real problem of myelofibrosis?
Anemia….no red cells
Morphology
Tear drop red cells
Masses of bone marrow in other organs are called?
Firohematopoeitic Extramedullary tumors…can basically be anywhere
