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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
Q

Hbg/Hct in P vera

A
  • elevated
26
Q

WBC in P vera

A
  • elevated
27
Q

platelets in P vera

A
  • elevated
28
Q

other important blood cell seen in P. vera

A
  • basophilia
29
Q

uric acid levels in P. vera

A
  • elevated
30
Q

epo levels in P. vera

A
  • low
31
Q

P. vera treatment

A
  • phlebotomy
  • hydroxyurea
  • low dose aspirin
32
Q

best treatment for P. vera

A
  • phlebotomy
33
Q

first thing in diagnosing essential thrombocytosis

A
  • rule out secondary causes of thrombocytosis
34
Q

important secondary cause of thrombocytosis

A
  • iron deficiency
35
Q

platelets numbers in ET

A

> 450 on 2 separate occasions

- at least one month apart

36
Q

ET progression to AML

A
  • rarely
37
Q

ET may progress to

A
  • secondary myelofibrosis
38
Q

ET major complication

A
  • thrombosis

- arterial or venous

39
Q

ET lab smear findings

A
  • large and bizarrely shaped platelets

- abnormal megakaryocytes in marrow

40
Q

who to treat for ET

A
  • people who have had or are at risk for thrombosis

- >65 years of age

41
Q

therapeutic agents for treatment of ET

A
  • hydroxyurea
42
Q

all myeloproliferative disorders can result in a

A
  • spent phase

- makes it difficult to distinguish from primary MF

43
Q

physical exam findings in MF

A
  • massive splenomegaly

- hepatomegaly

44
Q

peripheral smear of MF

A
  • leukoerythroblastic picture
  • tear drops
  • nucleated red blood cells
  • early granulocytes
45
Q

myelodysplastic syndrome definition

A
  • disordered maturation in 1 or more cell lines

- producing cytopenias

46
Q

population of dysplastic cells represent an

A
  • abnormal clone of cells
47
Q

MDS is a disease of which population

A
  • the elderly
48
Q

MDS peripheral cell abnoramalities

A
  • macrocytosis of RBCs
  • hypo granular or bi-lobed neutrophils
  • monocytosis
49
Q

marrow features of MDS

A
  • megaloblastic erythropoesis
  • ringed sideroblasts
  • dyserythropoiesis
  • small megakaryocytes with hypo lobed nuclei
50
Q

in MDS, blast cells should account for what percent of marrow cells

A
  • <20%
51
Q

what is dyserythropoiesis

A
  • abnormal nucleus of RBC precursors
52
Q

what are ringed sideroblasts

A
  • iron trapped in the mitochondria that rings the nuclei

- blue stain

53
Q

MDS cytogenetic abnormalities

A
  • monosomy 5, 7, 8
54
Q

deletion of which arms is associated with a better prognosis

A
  • long arm of 5
55
Q

treatment of MDS

A
  • supportive
  • transfusion of RBCs and platelets as necessary
  • growth factors
56
Q

certain agents that cause _____ in MDS treatment can lead to remission

A
  • DNA hypomethylation
57
Q

once the patient has developed AML in MDS,

A
  • the chances of achieving remission are decreased

- duration of any remission is much shorter

HEM Exam 2 (11 decks)

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