Term
What blast count is necessary for (almost) all cases of AML? |
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Definition
>20% (the ones that don't require >20% blasts are the genetic variants like inv(16)) |
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Term
What are the common clinical characteristics of AML? |
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Definition
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Term
As expression of CD34 increases in AML, what is it associated with? |
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Definition
More CD34 expression usually means poorer prognosis |
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Term
Prognostic vs Predictive biomarker |
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Definition
Prognostic = biomarker that info on the patients outcome; Predictive = marker that can identify a target for an intervention |
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Term
Treatment for AML M4 (monocytic) includes what drug combo? |
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Definition
Anthracycline + Cytarabine |
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Term
When is Complete Remission achieved with AML? |
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Definition
<5% bone marrow blasts and bone marrow aplasia |
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Term
Acute Promyelocytic Leukemia (APL) blasts appearance |
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Definition
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Term
What is the major threat of APL? |
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Definition
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Term
What should treatment for APL include? |
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Definition
FFP, Fibrinogen, and Platelets before giving ATRA |
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Term
What is the genetic rearrangement for APL t(15;17) |
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Definition
PML/RARA fusion resulting in promyelocyte undifferentiation unless given RARA |
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Term
When you suspect APL, when should you administer ATRA? |
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Definition
Right away, even before genetic testing is complete |
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Term
What drug can be given in conjunction with ATRA or if ATRA fails to treat APL? |
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Definition
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Term
What genetic abnormality is a poor prognostic predictor for AML? |
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Definition
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Term
What myeloid leukemia is most frequently associated with gingival hyperplasia? |
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Definition
M4/M5 Acute Myelomonocytic and Monoblastic Leukemia |
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Term
What translocation is commonly seen in AML (M4/M5)? |
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Definition
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Term
Acute Monoblastic Leukemia (M5) often shows extramedullary involvement where? |
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Definition
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Term
What are Pelger Huet cells and what genetic abnormality are they associated with? |
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Definition
Bi-lobed Neutrophils; del(7) aka Monosomy 7 |
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Term
What is Secondary AML is due to what most often and what does it result in? |
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Definition
Chemotherapy/Radiation resulting in Dysplasia of precursors in one or more cell lines |
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Term
Secondary AML usually involves what chromosomes? |
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Definition
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Term
How well does Seoncary AML respond to chemotherapy? |
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Definition
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Term
What are Myelodysplastic Syndromes characterized as? |
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Definition
Clonal disorders characterized by hypercelular marrows and Ineffective Hematopoiesis |
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Term
Bone marrow failure from Myelodysplastic Syndrome can cause what? |
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Definition
Infection/Bleeding and possible transformation to acute leukemia |
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Term
What are the characteristics and prognosis of Refractory Anemia MDS? |
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Definition
Reticulocytopenia with Erythroid Hyperplasia; Good prognosis |
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Term
What are the characteristics and prognosis of Refractory Anemia with Ring Sideroblasts MDS? |
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Definition
Same ase Refractory Anemia but with >15% ring Sideroblasts; Good prognosis |
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Term
What are the characteristics and prognosis of Refractory Cytopenias with Multileneage dysplasia MDS (dysplasia in more than 1 cell line) |
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Definition
<5% blasts in BM; Intermediate prognosis |
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Term
What are the characteristics and prognosis of Refractory anemia with Excess Blasts? |
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Definition
>5%,<20% Myeloblasts in BM; Poor prognosis |
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Term
What are the characteristics and prognosis of Chronic Myelomonocytic Leukemia MDS? |
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Definition
Dysplastic myeloproliferation with Peripheral blood Monocytosis; Poor prognosis |
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Term
What is the cause of Paroxysmal Nocturnal Hemoglobinuria (PNH)? |
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Definition
X-linked PIG-A mutation leading to defective CD59 and CD55 on RBCs, causing excess hemolysis of RBCs |
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Term
What treatment can be given for Myelodysplastic Syndromes? |
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Definition
Hematopoietic Colony Stimulating Factors (Erythropoietin, G-CSF, GM-CSF) |
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Term
What drug can be given to patients with a 5q deletion in MDS? |
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Definition
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Term
What two drugs interfere with the hypermethylation of DNA in MDS? |
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Definition
5-Azacytidine and Decitabine |
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Term
What mutation is responsible for >95% of Polycythemia Vera cases? |
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Definition
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Term
What is the treatment for Polycythemia Vera? |
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Definition
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Term
Treatment for PV if individuals are under 40 or pregnant is what? |
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Definition
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Term
Treatment for PV if individuals are over 40 is what? |
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Definition
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Term
What all can cause Secondary Polycythemia? |
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Definition
Chronic Hypoxemia (leads to increased Erythropoietin), or Erythropoietin-Secreting Tumor |
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Term
What is Budd-Chiari syndrome and what is it associated with? |
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Definition
Portal Vein Thrombosis associated with Essential Thrombocythemia |
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Term
Treatment for Thrombocythemia includes what? |
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Definition
Anything to reduce platelet counts; Hydroxyurea, Anagrelide, Interferon |
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Term
What is Erythromelalgia and what is associated with the condition, what is the treatment? |
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Definition
Burning, aching thrombing pain especially in lower extremities that is due to ET; give low dose aspirin for treatment |
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Term
What type of blood smear does Myelofibrosis show (particular name)? |
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Definition
Myelophthisic (NRBCs and Granulocyte precursors, Teardrops) |
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Term
________ is a common end-result in the marrow of Myeloproliferative diseases |
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Definition
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Term
Why would a Splenectomy be performed in Myelofibrosis cases? |
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Definition
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