Ipapburke Hematology Test 2- Myeloproliferative

Front 1

Definition of myeloproliferative

Back 1

excessive number of cells in the myeloid line

Front 2

Definition of myelodysplasia

Back 2

deformed or ineffective cells

Front 3

Definition of myelofibrosis

Back 3

bone marrow fibrosis or scarring

Front 4

Definition of myelophthisis

Back 4

Secondary myelofibrosis

Front 5

What do clonal disorders of hematopoietic stem cells result in?

Back 5

overproduction of various cell lines

Front 6

Despite the fact that there is stem cell line predominance in clonal disorders, ________ cell lines can be __________.

Back 6

all; affected

Front 7

What accounts for the possibility of any myeloproliferative disorders to progress to Acute Myelogenous Leukomia?

Back 7

shared origin

Front 8

What condition has the highest risk for AML?

Back 8

Chronic Myelogenous Leukemia

Front 9

Lowest risk to progress to AML

Back 9

essential thrombocytosis

Front 10

PV transforms to what two conditions

Back 10

AML and myelofibrosis

Front 11

Myelofibrosis starts are one of what 2 conditions

Back 11

PV or ET

Front 12

What is the role of the PA in oncology?

Back 12

recognition of overproliferation

Front 13

Another name for Bone Marrow Transplant

Back 13

SCT (Stem Cell Transplant)

Front 14

Stem cells for bone marrow transplant are harvested from (3)

Back 14

donor bone marrow under general anesthesia; peripheral blood via apheresis; umbilical cord blood via aspiration

Front 15

What is an "allogenic" source of stem cells for bone marrow transplant?

Back 15

from HLA matched individual

Front 16

What is an "syngeneic" source of stem cells for bone marrow transplant?

Back 16

from identical twin

Front 17

What is the most ideal source for stem cells for bone marrow transplant?

Back 17

syngeneic

Front 18

What is an "autologous" source of stem cells for bone marrow transplant?

Back 18

from the patient (filtered w/ sophisticated apheresis)

Front 19

What is the benefit of autologous source of stem cells for bone marrow transplant?

Back 19

less risk for GVHD

Front 20

What is the increased risk of autologous source of stem cells for bone marrow transplant?

Back 20

increased chance of tumor contamination

Front 21

What is a preparative regimen (general consideration for bone marrow transplant)?

Back 21

method of tx involving potent chemo and/or radiation w/ the goal of eradicating the underlying dz after stem cells are harvested

Front 22

What does preparative regimen usually result in?

Back 22

complicated and prolonged cytopenias from bone marrow toxicity

Front 23

Why is there a persistent risk of relapse with most malignancies even after intensive preparative regimens?

Back 23

some normal and malignant stem cells will likely retain resistance to even most intensive preparative regimens

Front 24

Missing an affected stem cell during the apheresis process can account for _________.

Back 24

relapse

Front 25

What do we do after preparative regimen in malignancy?

Back 25

after therapy, donor stem cells are given back to the patient via peripheral blood transfusion followed by careful immunological monitoring (strive for 5 years cancer free)

Front 26

What leads to unregulated cell overproduction in Chronic Myelogenous Leukemia?

Back 26

increased tyrosine kinase activity (caused by the Philadelphia Chromosome)

Front 27

This is a condition caused by Chromosome 9:22 Reciprocal Translocation resulting in the bcr-abl gene (oncogene)

Back 27

Chronic Myelogenous Leukemia

Front 28

What chromosome is ABL (abelson)?

Back 28

chromosome 9

Front 29

What chromosome is BCR (breakpoint cluster region)?

Back 29

chromosome 22

Front 30

Another name for Chromosome 9:22 Reciprocal Translocation

Back 30

bcr-abl

Front 31

bcr-abl is known as?

Back 31

Philadelphia Chromosome

Front 32

Median age at diagnosis of CML

Back 32

65

Front 33

What association can be made to a demographic group for CML?

Back 33

no definitive hereditary, familial, geographic, ethnic, or economic associations

Front 34

Characteristic symptoms of CML

Back 34

fatigue, anorexia, weight loss, peripheral blood findings, marrow findings

Front 35

Peripheral blood findings in CML

Back 35

elevated WBC ct, elevated platelet count in 30-50%, basophilia, reduced leukocyte alkaline phosphatase activity, all stage of granulocyte differentiation visible on peripheral smear

Front 36

Bone marrow findings in CML

Back 36

hypercellularity, reduced fat content, increased ratio of myeloid to erythroid cells, increased megakaryocytes, blasts and promyelocytes constitute less than 10% of cells

Front 37

What distinguishs the phases of Chronic Myelogenous Leukemia?

Back 37

distinguished by the number of blasts present in peripheral blood or bone marrow

Front 38

10% blasts on peripheral smear- what stage of CML?

Back 38

chronic

Front 39

15% blasts on peripheral smear- what stage of CML?

Back 39

accelerated

Front 40

20% blasts on peripheral smear- what stage of CML?

Back 40

acute

Front 41

Another name for acute Myelogenous Leukemia

Back 41

blast phase

Front 42

What stage are most patients diagnosed with Chronic Myelogenous Leukemia?

Back 42

in the chronic phase, but usually progress to the acute phase within 3-5 years without treatment

Front 43

What is the annual risk of conversion of CML to AML?

Back 43

3-4%

Front 44

How long will a patient survive after conversion to the acute myelogenous leukemia?

Back 44

3-6 months with treatment

Front 45

treatment for Chronic Myelogenous Leukemia

Back 45

Imatinib (Gleevec)--inhibits tyrosine kinase activity of bcr-abl gene---lifelong therapy

Front 46

What is the only curative therapy for CML?

Back 46

bone marrow transplant w/ bonus graft versus leukemia effect with recurrence

Front 47

What is the pathophysiology of Polycythemia Vera related to?

Back 47

increased blood viscosity

Front 48

Besides overproduction of RBCs in polycythemia, what else may occur?

Back 48

leukocytosis, thrombocystosis (same stem cell line) and splenomegaly

Front 49

Average age at diagnosis for PV

Back 49

60

Front 50

Major criteria for PV diagnosis

Back 50

Increased red cell mass (males >36, women >32), arterial O2 sat >92%, splenomegaly)

Front 51

Minor criteria for PV diagnosis

Back 51

Platelet count greater than 400,000, WBC >12,000, LAP score >100, serum B12 >900

Front 52

LAP score in CML vs. PV

Back 52

LAP score low in CML, high in PV

Front 53

Clinical findings in PV

Back 53

erythromelalgia, post shower pruritis, paradoxical bleeding risk, thrombotic complications

Front 54

What is erythromelalgia?

Back 54

burning feet and lower leg pain

Front 55

What is post shower/bath pruritis?

Back 55

heat activated mast cells

Front 56

What is the cause of paradoxical bleeding risk in PV?

Back 56

dysfunctional platelets

Front 57

What thrombotic complications are found in polycythemia vera?

Back 57

stroke, MI, and mesenteric ischemia in 20-30%

Front 58

H and H in PV lab findings

Back 58

elevated

Front 59

Leukocyte Alkaline Phosphatase level in PV

Back 59

elevated

Front 60

Erythropoietin levels in PV

Back 60

low (It is not a O2 issue)---distinguishing feature

Front 61

More common signs and symptoms in PV

Back 61

plethora, pruritis after bathing, splenomegaly, weight loss, weakness, sweating

Front 63

Suspect hemoglobin and hematocrit values in PV

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hematocrit more than 52% in white men, more than 47% in blacks and women; HgB over 18 in white men, over 16 in blacks and women

Front 64

Less common symptoms of PV

Back 64

bruising, epistaxis, Budd Chiari, erythromelalgia, gout, hemorrhagic events, hepatomegaly, ischemic fingers, thrombotic events, transient neurologic complaints, atypical chest pain

Front 65

Explain polycythemia with a spurious cause.

Back 65

severe volume depletion for any reason (heat injury, diuretic use, severe diarrhea, etc)

Front 66

Explain polycythemia that is hypoxia driven.

Back 66

COPD, high altitude, carbon monoxide exposure, hypoventilation---erythropoietin dependent

Front 67

Explain polycythemia that is hypoxia independent.

Back 67

malignant tumors like renal cell carcinoma, polycystic kidney disease, blood doping----pathologic erythropoietin production

Front 68

Treatment for PV

Back 68

phlebotomy to keep Hct <45 or as symptoms and risk dictate; Hydroxyurea and aspirin

Front 69

Prognosis for PV

Back 69

untreated live 6 to 18 mths; treated patients 9-13 years depending on therapy

Front 70

Primary causes of death in polycythemia vera patients.

Back 70

thrombotic complications, hematologic malignancies, hemorrhagic complications, other causes about 45%

Front 71

This condition is predominantly platelet overproduction from proliferating megakaryocytes

Back 71

Essential thrombocytosis (ET)

Front 72

Median age at diagnosis of ET

Back 72

50, only 20% under 40

Front 73

What is the relationship of ET to thrombopoietin abnormalities?

Back 73

no clear relationship (there is also no evidence of unique or common clonal abnormality or hereditary predisposition)

Front 74

What does the diagnostic focus for ET focus on?

Back 74

exclusion of other MPDs as well as other causes of reactive thrombocytosis

Front 75

Major categories of causes of reactive thrombocytosis

Back 75

nonmalignant hematologic coditions, malignant conditions, acute and chronic inflammatory conditions, tissue damage, infections, exercise, allergic reactions, chronic renal disease

Front 76

Platelets in ET will be what count?

Back 76

consistently greater than 600,000

Front 77

Diagnostic criteria for ET (all exclusionary)

Back 77

platelets over 600,000 always **, megakaryocyte hyperplasia on biopsy, no Philadelphia chromosome, no other cause of reactive thrombocytosis, no MDS or myelofibrosis, normal iron stores

Front 78

Classical features of ET

Back 78

vasomotor symptoms (HA, syncope, atypical chest pain, acral parasthesia, livedo reticularis, erythromelalgia), thrombotic risk, paradoxical bleeding risk

Front 79

Tx for ET

Back 79

keep platelet count under 500,000, low dose aspirin, hydroxyurea, analgrelide (interferon), life- threatening situations- platelet apheresis

Front 80

How do you keep platelet count under 500,000 in ET?

Back 80

apheresis

Front 81

What is the purpose of aspirin in ET?

Back 81

used to reduce risk of thrombosis--keeps platelets slippery

Front 82

What is the risk of using aspirin in ET?

Back 82

there may an increased risked of bleeding if aspirin is initiated while the platelet count is very high

Front 83

What is another name of Agnogenic Myeloid Metaplasia?

Back 83

Myelofibrosis

Front 84

What characterizes Myelofibrosis?

Back 84

bone marrow fibrosis and thrombocytopenia

Front 85

What is the primary abnormality in Myelofibrosis?

Back 85

abnormal, excessive megakaryocytes that cause secondary bone marrow fibrosis due to overproduction of collagen from fibroblasts

Front 86

S/S of myelofibrosis

Back 86

abdominal fullness (splenomegaly), bone pain, bruising and easy bleeding (few platelets), fatigue, increased susceptibility to platelets, pallor and shortness of breath (anemia)

Front 87

Least common MPD

Back 87

Myelofibrosis

Front 88

What is the cause of pallor and shortness of breath in Myelofibrosis?

Back 88

anemia

Front 89

What do people with Myelofibrosis bruise and bleed easily?

Back 89

few platelets

Front 90

Why is there splenomegaly with Myelofibrosis?

Back 90

due to extramedullary hematopoiesis

Front 91

What is a end result of myelofibrosis?

Back 91

eventually burns out the bone marrow and results in predominantly extramedullary hematopoiesis

Front 92

What is the cause of severe abdominal pain crises in Myelofibrosis?

Back 92

due to ischemic in GI tract

Front 93

Why are there too "few" platelets in Myelofibrosis?

Back 93

eventually bone marrow burns out, as well as spleen taking up dysfunctional cells

Front 94

What is found on a leukoerythroblastic peripheral blood smear?

Back 94

immature WBCs, nucleated RBCs, tear drop RBCs, and thrombocytosis

Front 95

Tx for myelofibrosis

Back 95

bone marrow transplant, splenectomy, radiatin therapy (splenic irradiation), drugs (anagrelide, hydroxyurea, and thalidomide)

Front 96

Prognosis for Myelofibrosis

Back 96

It progresses slowly so people may live for 10 years or longer. Outcomes are determined by how well bone marrow functions.