Myeloid Neoplasms discreet facts Flashcards
Chemotherapeutic agent that induces DNA mutations that can be related to development of hematopioetic neoplasms
Alkylating agents
also Topoisomerase Inhibitors (much faster rate of development after chemo)
Type of receptor that underlies genetic disorders of myeloproliferative neoplasms?
Tyrosine Kinase
Tyrosine kinase inhibitors treat Chronic Myeloid Leukemias however patients whose CML have developed a mutation within the ________ will not respond to the TKIs though 2nd and 3rd generation TKIs overcome these mutations?
in the ATP binding pocket that prevents drug from binding and also
Accelerated phase of CML can result in blast crisis which is a sudden and catastrophic ability to mature. The 2 types of Blast crisis is
70% Acute Myeloid Leukemia
30% is Acute Lymphoid Leukemia
This indicates that CML comes from pluripotential stem cell
Point mutation in pseudokinase domain of ______ involved in Polycythemia Vera
JAK2
JAK2 inhibitor that is pretty toxic
Ruxonitib
Presentation for early stages of Polycythemia Vera
Increased RBC mass: plethora, headache, dizziness, angina, visual disturbances, intermitted claudication (pain in walking)
normochromic, normocytic RBCs until later in the disease
low or normal serum EPO
What lab value would help to distinguish Polycythemia vera from a reactive polycythemia?
low or normal serum EPO or JAK2 mutation
Treatment of Polycythemia
Vera
Phlebotomy
Thrombopoietin receptor mutated in Essential Thrombocytosis or Primary Myeloproliferative disorder
JAK2
Calreticulin
Tear drop shaped RBCs
RBCs that have passed through areas of fibrosis in the bone marrow
Extramedullary hematopoeisis leading to _________ from Primary Myelofibrosis
Splenomegaly
How do you distinguish between reactive thrombocytosis or essential thrombocytosis (neoplastic)
Find a clonal abnormality to dx essential thrombocytosis
Mastocytosis mutation
c-Kit
Localized cutaneous manifestation of Mastocytosis?
Urticaria Pigmentosa
which myeloproliferative neoplasm has erythroid hyperplasia
polycythemia vera
which myeloproliferative neoplasm has Jak2 mutation?
Polycythemia vera
Primary myelofibrosis
Essential Thrombocytosis
which myeloproliferative neoplasm has BCR/ABL fusion
Chronic Myeloid Leukemia
c-Kit mutation
Mastocytosis
Markedly increased platelets
Essential thrombocytosis
Key morphology of acute leukemia
more than 20% myeloid cells in the bone marrow
Drug association with Acute myeloid leukemia
Alkylating agents (very long lag time)
Topoisomerase inhibitors (short lag time)
Trisomy assoc with Acute Myeloid Leukemia
Trisomy 21
Acute Myeloid Leukemia is not one disease because it is the conglomeration of a bunch of genetic aberrations. It presents with
Anemia
Thrombocytopenia
Hepatosplenomegaly
