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Myeloid Disorders
Lecture 9/30
42
Medical
Professional
09/30/2013

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Term
What blast count is necessary for (almost) all cases of AML?
Definition
>20% (the ones that don't require >20% blasts are the genetic variants like inv(16))
Term
What are the common clinical characteristics of AML?
Definition
Pancytopenia, Bone pain
Term
As expression of CD34 increases in AML, what is it associated with?
Definition
More CD34 expression usually means poorer prognosis
Term
Prognostic vs Predictive biomarker
Definition
Prognostic = biomarker that info on the patients outcome; Predictive = marker that can identify a target for an intervention
Term
Treatment for AML M4 (monocytic) includes what drug combo?
Definition
Anthracycline + Cytarabine
Term
When is Complete Remission achieved with AML?
Definition
<5% bone marrow blasts and bone marrow aplasia
Term
Acute Promyelocytic Leukemia (APL) blasts appearance
Definition
Hypergranular
Term
What is the major threat of APL?
Definition
Consumptive Coagulopathy
Term
What should treatment for APL include?
Definition
FFP, Fibrinogen, and Platelets before giving ATRA
Term
What is the genetic rearrangement for APL t(15;17)
Definition
PML/RARA fusion resulting in promyelocyte undifferentiation unless given RARA
Term
When you suspect APL, when should you administer ATRA?
Definition
Right away, even before genetic testing is complete
Term
What drug can be given in conjunction with ATRA or if ATRA fails to treat APL?
Definition
Arsenic Trioxide
Term
What genetic abnormality is a poor prognostic predictor for AML?
Definition
Monosomy 7 or 5
Term
What myeloid leukemia is most frequently associated with gingival hyperplasia?
Definition
M4/M5 Acute Myelomonocytic and Monoblastic Leukemia
Term
What translocation is commonly seen in AML (M4/M5)?
Definition
11q23
Term
Acute Monoblastic Leukemia (M5) often shows extramedullary involvement where?
Definition
Meningeal Involvement
Term
What are Pelger Huet cells and what genetic abnormality are they associated with?
Definition
Bi-lobed Neutrophils; del(7) aka Monosomy 7
Term
What is Secondary AML is due to what most often and what does it result in?
Definition
Chemotherapy/Radiation resulting in Dysplasia of precursors in one or more cell lines
Term
Secondary AML usually involves what chromosomes?
Definition
5,7, or 11q23
Term
How well does Seoncary AML respond to chemotherapy?
Definition
Poorly
Term
What are Myelodysplastic Syndromes characterized as?
Definition
Clonal disorders characterized by hypercelular marrows and Ineffective Hematopoiesis
Term
Bone marrow failure from Myelodysplastic Syndrome can cause what?
Definition
Infection/Bleeding and possible transformation to acute leukemia
Term
What are the characteristics and prognosis of Refractory Anemia MDS?
Definition
Reticulocytopenia with Erythroid Hyperplasia; Good prognosis
Term
What are the characteristics and prognosis of Refractory Anemia with Ring Sideroblasts MDS?
Definition
Same ase Refractory Anemia but with >15% ring Sideroblasts; Good prognosis
Term
What are the characteristics and prognosis of Refractory Cytopenias with Multileneage dysplasia MDS (dysplasia in more than 1 cell line)
Definition
<5% blasts in BM; Intermediate prognosis
Term
What are the characteristics and prognosis of Refractory anemia with Excess Blasts?
Definition
>5%,<20% Myeloblasts in BM; Poor prognosis
Term
What are the characteristics and prognosis of Chronic Myelomonocytic Leukemia MDS?
Definition
Dysplastic myeloproliferation with Peripheral blood Monocytosis; Poor prognosis
Term
What is the cause of Paroxysmal Nocturnal Hemoglobinuria (PNH)?
Definition
X-linked PIG-A mutation leading to defective CD59 and CD55 on RBCs, causing excess hemolysis of RBCs
Term
What treatment can be given for Myelodysplastic Syndromes?
Definition
Hematopoietic Colony Stimulating Factors (Erythropoietin, G-CSF, GM-CSF)
Term
What drug can be given to patients with a 5q deletion in MDS?
Definition
Lenalidomide
Term
What two drugs interfere with the hypermethylation of DNA in MDS?
Definition
5-Azacytidine and Decitabine
Term
What mutation is responsible for >95% of Polycythemia Vera cases?
Definition
V617F
Term
What is the treatment for Polycythemia Vera?
Definition
Phlebotomy
Term
Treatment for PV if individuals are under 40 or pregnant is what?
Definition
alpha interferon
Term
Treatment for PV if individuals are over 40 is what?
Definition
Hydroxyurea
Term
What all can cause Secondary Polycythemia?
Definition
Chronic Hypoxemia (leads to increased Erythropoietin), or Erythropoietin-Secreting Tumor
Term
What is Budd-Chiari syndrome and what is it associated with?
Definition
Portal Vein Thrombosis associated with Essential Thrombocythemia
Term
Treatment for Thrombocythemia includes what?
Definition
Anything to reduce platelet counts; Hydroxyurea, Anagrelide, Interferon
Term
What is Erythromelalgia and what is associated with the condition, what is the treatment?
Definition
Burning, aching thrombing pain especially in lower extremities that is due to ET; give low dose aspirin for treatment
Term
What type of blood smear does Myelofibrosis show (particular name)?
Definition
Myelophthisic (NRBCs and Granulocyte precursors, Teardrops)
Term
________ is a common end-result in the marrow of Myeloproliferative diseases
Definition
Fibrosis
Term
Why would a Splenectomy be performed in Myelofibrosis cases?
Definition
Pain relief only
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