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HEM Exam 2 > Myeloproliferative and Myelodysplastic > Flashcards

Myeloproliferative and Myelodysplastic Flashcards

1
Q

erythrocytosis definition

A
  • increase in number of circulating RBCs per volume of blood
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2
Q

synonym for erythrocytosis

A
  • polycythemia
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3
Q

hemoglobin levels in erythrocytosis

A
  • elevated
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4
Q

hematocrit in erythrocytosis

A
  • elevated
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5
Q

symptoms of hyper viscosity syndrome

A
  • headache
  • visual changes
  • tinnitus
  • dizziness
  • paresthesias
  • decreased mental acuity
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6
Q
  • treatment of erythrocytosis with hyper viscosity symptoms
A
  • phlebotomy
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7
Q

secondary erythrocytosis due to

A
  • tissue hypoxia inducing epo production by the kidney
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8
Q

risk of blood clots in secondary erythrocytosis

A
  • no
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9
Q

big things that cause tissue hypoxia

A
  • obstructive sleep apnea

- high carboxyhemoglobin levels

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10
Q

less common cause of secondary erythrocytosis

A
  • treatment with erythropoietin or androgens

- tumors of liver or kidney that secrete epo

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11
Q

the myeloproliferative disorders

A
  • P. vera
  • essential thrombocythemia
  • myelofibrosis
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12
Q

myeloproliferative disorders are what kind of disorders

A
  • stem cell disorders
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13
Q

myeloproliferative disorders lead to

A
  • autonomous production of hematopoietic cells from all three lineages (white cells, red cells, platelets)
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14
Q

all myeloproliferative disorders are

A
  • clonal
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15
Q

P. vera mostly seen in which population

A
  • age 50-75
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16
Q

importance of erythroid progenitor cells of patients with P. vera

A
  • grow and divide in absence of erythropoietin
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17
Q

diagnosis of P. vera

A
  • low or undetectable epo

- JAK2 mutation

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18
Q

JAK2 mutation in P. vera

A
  • JAK2 V617F
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19
Q

P. vera phases

A
  • latent phase
  • proliferative phase
  • spent phase
  • secondary AML
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20
Q

latent phase of P. vera

A
  • asymptomatic
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21
Q

proliferative phase of P. vera

A
  • hyper metabolic

- symptoms of hyperviscosity or thrombosis

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22
Q

spent phase of P. vera

A
  • anemia
  • leukopenia
  • secondary myelofibrosis
  • increased liver and spleen size
  • fevers
  • weight loss

ALS LSFW

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23
Q

symptoms specific to P. vera and myeloproliferative diseases

A
  • pruritus after bathing
  • erythromelalgia
  • hyper metabolic symptoms
  • thrombosis (Budd chiari syndrome)
  • hemorrhage

HEPTH

24
Q

P vera physical exam findings

A
  • splenomegaly
  • facial plethora
  • hepatomegaly
25
Hbg/Hct in P vera
- elevated
26
WBC in P vera
- elevated
27
platelets in P vera
- elevated
28
other important blood cell seen in P. vera
- basophilia
29
uric acid levels in P. vera
- elevated
30
epo levels in P. vera
- low
31
P. vera treatment
- phlebotomy - hydroxyurea - low dose aspirin
32
best treatment for P. vera
- phlebotomy
33
first thing in diagnosing essential thrombocytosis
- rule out secondary causes of thrombocytosis
34
important secondary cause of thrombocytosis
- iron deficiency
35
platelets numbers in ET
> 450 on 2 separate occasions | - at least one month apart
36
ET progression to AML
- rarely
37
ET may progress to
- secondary myelofibrosis
38
ET major complication
- thrombosis | - arterial or venous
39
ET lab smear findings
- large and bizarrely shaped platelets | - abnormal megakaryocytes in marrow
40
who to treat for ET
- people who have had or are at risk for thrombosis | - >65 years of age
41
therapeutic agents for treatment of ET
- hydroxyurea
42
all myeloproliferative disorders can result in a
- spent phase | - makes it difficult to distinguish from primary MF
43
physical exam findings in MF
- massive splenomegaly | - hepatomegaly
44
peripheral smear of MF
- leukoerythroblastic picture - tear drops - nucleated red blood cells - early granulocytes
45
myelodysplastic syndrome definition
- disordered maturation in 1 or more cell lines | - producing cytopenias
46
population of dysplastic cells represent an
- abnormal clone of cells
47
MDS is a disease of which population
- the elderly
48
MDS peripheral cell abnoramalities
- macrocytosis of RBCs - hypo granular or bi-lobed neutrophils - monocytosis
49
marrow features of MDS
- megaloblastic erythropoesis - ringed sideroblasts - dyserythropoiesis - small megakaryocytes with hypo lobed nuclei
50
in MDS, blast cells should account for what percent of marrow cells
- <20%
51
what is dyserythropoiesis
- abnormal nucleus of RBC precursors
52
what are ringed sideroblasts
- iron trapped in the mitochondria that rings the nuclei | - blue stain
53
MDS cytogenetic abnormalities
- monosomy 5, 7, 8
54
deletion of which arms is associated with a better prognosis
- long arm of 5
55
treatment of MDS
- supportive - transfusion of RBCs and platelets as necessary - growth factors
56
certain agents that cause _____ in MDS treatment can lead to remission
- DNA hypomethylation
57
once the patient has developed AML in MDS,
- the chances of achieving remission are decreased | - duration of any remission is much shorter

HEM Exam 2 (11 decks)

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